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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

    International Nuclear Information System (INIS)

    Haider, E.; Raja, S.; Wali, W.; Tariq, M.

    2013-01-01

    A case of 50 years of age, male with sporadic Creutzfeldt Jakob Disease (sCJD) is reported. Patient had dementia, behavioural abnormalities, unsteady gait and myoclonic jerks. Magnetic resonance imaging (MRI) brain T2 weighted and Fluid Attenuated Inverse Recovery (FLAIR) images showed abnormally increased signal intensity in caudate nucleus and putamen. Scalp electroencephalogram (EEG) revealed periodic synchronous biphasic sharp wave complexes. On the basis of history, clinical findings, typical MRI brain and EEG changes, diagnosis of sporadic CJD was made. (author)

  3. 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.

  4. A test for Creutzfeldt-Jakob disease using nasal brushings.

    Science.gov (United States)

    Orrú, Christina D; Bongianni, Matilde; Tonoli, Giovanni; Ferrari, Sergio; Hughson, Andrew G; Groveman, Bradley R; Fiorini, Michele; Pocchiari, Maurizio; Monaco, Salvatore; Caughey, Byron; Zanusso, Gianluigi

    2014-08-07

    Definite diagnosis of sporadic Creutzfeldt-Jakob disease in living patients remains a challenge. A test that detects the specific marker for Creutzfeldt-Jakob disease, the prion protein (PrP(CJD)), by means of real-time quaking-induced conversion (RT-QuIC) testing of cerebrospinal fluid has a sensitivity of 80 to 90% for the diagnosis of sporadic Creutzfeldt-Jakob disease. We have assessed the accuracy of RT-QuIC analysis of nasal brushings from olfactory epithelium in diagnosing sporadic Creutzfeldt-Jakob disease in living patients. We collected olfactory epithelium brushings and cerebrospinal fluid samples from patients with and patients without sporadic Creutzfeldt-Jakob disease and tested them using RT-QuIC, an ultrasensitive, multiwell plate-based fluorescence assay involving PrP(CJD)-seeded polymerization of recombinant PrP into amyloid fibrils. The RT-QuIC assays seeded with nasal brushings were positive in 30 of 31 patients with Creutzfeldt-Jakob disease (15 of 15 with definite sporadic Creutzfeldt-Jakob disease, 13 of 14 with probable sporadic Creutzfeldt-Jakob disease, and 2 of 2 with inherited Creutzfeldt-Jakob disease) but were negative in 43 of 43 patients without Creutzfeldt-Jakob disease, indicating a sensitivity of 97% (95% confidence interval [CI], 82 to 100) and specificity of 100% (95% CI, 90 to 100) for the detection of Creutzfeldt-Jakob disease. By comparison, testing of cerebrospinal fluid samples from the same group of patients had a sensitivity of 77% (95% CI, 57 to 89) and a specificity of 100% (95% CI, 90 to 100). Nasal brushings elicited stronger and faster RT-QuIC responses than cerebrospinal fluid (PCreutzfeldt-Jakob disease and indicated substantial prion seeding activity lining the nasal vault. (Funded by the Intramural Research Program of the National Institute of Allergy and Infectious Diseases and others.).

  5. Creutzfeldt-Jakob Disease and Psychiatric Symptoms

    Directory of Open Access Journals (Sweden)

    Soner Cakmak

    2013-08-01

    Full Text Available Creutzfeldt-Jakob disease is a rapidly progressive, degenerative slow virus infection disease of central nervous system. Based on etiologic origins, four different Creutzfeldt-Jakob disease subtypes have been identified: sporadic, genetic, iatrogenic and variant. The clinical course generally begin with apathy, irritability, behavioral changes, speech problems, memory deterioration, rapidly progresses and concludes with death over a period of 3-12 months. Symptoms are observed secondary to brain cortex, cerebellum, corticospinal tracts, spinal anterior horn cells and basal ganglia damage. Unusual (%5-10 cases can survive up to 2 years. The initial symptoms of disease can be sudden which resultsin adjustment problems leading patients to seek psychiatric help. Patients could receive different diagnosis such as psychosis, depression with psychotic features, and treatments at this stages. Early diagnosis is crucial because of management of the disease and treatment approaches. In this article diagnosis and clinical features of Creutzfeldt-Jakob Disease and related psychiatric symptoms have been briefly reviewed. [Archives Medical Review Journal 2013; 22(4.000: 631-643

  6. Variant Creutzfeldt-Jakob Disease (vCJD)

    Science.gov (United States)

    ... Form Controls Cancel Submit Search the CDC Variant Creutzfeldt-Jakob Disease (vCJD) Note: Javascript is disabled or is not ... gov . Recommend on Facebook Tweet Share Compartir Variant Creutzfeldt-Jakob disease (vCJD) is a prion disease that was first ...

  7. Treating seizures in Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Ng, Marcus C; Westover, M Brandon; Cole, Andrew J

    2014-01-01

    Seizures are known to occur in Creutzfeldt-Jakob disease (CJD). In the setting of a rapidly progressive condition with no effective therapy, determining appropriate treatment for seizures can be difficult if clinical morbidity is not obvious yet the electroencephalogram (EEG) demonstrates a worrisome pattern such as status epilepticus. Herein, we present the case of a 39-year-old man with CJD and electrographic seizures, discuss how this case challenges conventional definitions of seizures, and discuss a rational approach toward treatment. Coincidentally, our case is the first report of CJD in a patient with Stickler syndrome.

  8. Sporadic Creutzfeldt-Jakob Disease (sCJD)

    Centers for Disease Control (CDC) Podcasts

    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.

  9. Radiological assessment of Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  10. Radiological assessment of Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Tschampa, Henriette J.; Urbach, Horst; Zerr, Inga

    2007-01-01

    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.)

  11. MRI of sporadic Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Kong, A.; Vliet, A. Van der.

    2008-01-01

    Full text: The key MRI findings in five cases of sporadic Creutzfeldt-Jakob disease (CJD) are illustrated with four 'definite' and one 'probable' according to World Health Organization criteria. Close attention to fluid-attenuation inversion recovery and diffusion-weighted imaging sequences are important for diagnosis, noting especially restricted diffusion in cortical and deep grey matter. Our study and those of others show predominant cortical, caudate and thalamic involvement. This pattern is highly sensitive and specific for the diagnosis. Fluid-attenuation inversion recovery and diffusion-weighted imaging signal abnormality becomes progressively more extensive and bilateral as disease progresses, but may become less pronounced in end-stage disease because of atrophy.

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

    Science.gov (United States)

    ... here Home » Disorders » Patient & Caregiver Education » Fact Sheets Creutzfeldt-Jakob Disease Fact Sheet for Healthcare Workers and Morticians Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain disorder that ...

  13. 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.

  14. Rapid cognitive decline: not always Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Randall, A; Ellis, R; Hywel, B; Davies, R R; Alusi, S H; Larner, A J

    2015-01-01

    A patient with rapidly progressive cognitive decline over an approximately four month period was suspected to have sporadic Creutzfeldt-Jakob disease. Features thought to support this diagnosis included psychiatric symptoms (anxiety and depression), visual hallucinations and a visual field defect. However, the finding of papilloedema broadened the differential diagnosis. Although standard brain imaging and electroencephalography had shown only non-specific abnormalities, subsequent cerebral angiography disclosed an intracranial dural arteriovenous fistula. Following embolisation, the patient made a good functional recovery. Intracranial dural arteriovenous fistula merits consideration in any patient with subacute cognitive decline, and should be included in the differential diagnosis of sporadic Creutzfeldt-Jakob disease.

  15. Sequential MRI in a case of Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Tribl, G.G.; Zeitlhofer, J.; Asenbaum, S.; Wessely, P.; Strasser, G.; Prayer, D.; Jarius, C.

    2002-01-01

    A 48-year-old man suddenly developed clinically and electroencephalographically nonspecific dementia. On MRI sequences, only diffusion-weighted images (DWI) of the cortex were unequivocally pathological. Obvious atrophy and basal ganglia signal changes appeared only 9 months after the onset. Brain biopsy confirmed Creutzfeldt-Jakob disease (CJD). In rapidly progressive dementia, we recommend DWI for early diagnosis of CJD. (orig.)

  16. Pathologically confirmed autoimmune encephalitis in suspected Creutzfeldt-Jakob disease

    NARCIS (Netherlands)

    Maat, P.; de Beukelaar, J.W.; Jansen, C.; Schuur, M.; van Duijn, C.M.; van Coevorden, M.H.; de Graaff, E.; Titulaer, E.; Rozemuller, A.J.M.; Sillevis Smitt, P.

    2015-01-01

    Objective: To determine the clinical features and presence in CSF of antineuronal antibodies in patients with pathologically proven autoimmune encephalitis derived from a cohort of patients with suspected Creutzfeldt-Jakob disease (CJD). Methods: The Dutch Surveillance Centre for Prion Diseases

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

    NARCIS (Netherlands)

    I. Zerr; K. Kallenberg; D.M. Summers; C. Romero; A. Taratuto; U. Heinemann; M. Breithaupt; D. Varges; B. Meissner; A. Ladogana (Anna); M. Schuur (Maaike); S. Haik; S.J. Collins (Steven); G.H. Jansen (Gerard); G.B. Stokin; J. Pimentel; E. Hewer; D. Collie; P. Smith; H. Roberts; J.P. Brandel; P. Tikka-Kleemola (Päivi); M. Pocchiari (Maurizio); C. Begue; P. Cras (Patrick); R.G. Will; P. Sanchez-Juan (Pascual)

    2009-01-01

    textabstractSeveral 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

  18. Creutzfeldt-Jakob disease in Venezuela a case report

    Directory of Open Access Journals (Sweden)

    Alejandro J. Caraballo H.

    1991-06-01

    Full Text Available A case of Creutzfeldt-Jakob disease (CJD in a 32 year old man is presented. The clinical picture included a rapid progressive dementia associated with ataxia, global aphasia, myoclonus and pyramidal signs, death ocurred after 13 months. The diagnosis of CJD was confirmed by CT and neuropathological studies. This is the first report of CJD occurring in Venezuela.

  19. Topodiagnosis in Creutzfeldt-Jakob disease by HMPAO-SPECT

    International Nuclear Information System (INIS)

    Heye, N.; Farahati, J.; Heinz, A.; Buettner, T.; Przuntek, H.; Reiners, C.

    1993-01-01

    A 80-year old female presented with an early stage of Creutzfeldt-Jakob disease with clinical, neurophysiological and neuropathological findings suggesting a focal involvement of the brain. HMPAO SPECT disclosed asymmetries of regional cerebral perfusion, thus suggesting that it may be a further diagnostic instrument in this disease. (orig.) [de

  20. Sequential MRI in a case of Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Tribl, G.G.; Zeitlhofer, J.; Asenbaum, S.; Wessely, P. [Universitaetsklinik fuer Neurologie, Allgemeines Krankenhaus Wien (Austria); Strasser, G.; Prayer, D. [Universitaetsklinik fuer Radiodiagnostik, Allgemeines Krankenhaus Wien (Austria); Jarius, C. [Klinisches Institut fuer Neurologie, Universitaet Wien (Austria)

    2002-03-01

    A 48-year-old man suddenly developed clinically and electroencephalographically nonspecific dementia. On MRI sequences, only diffusion-weighted images (DWI) of the cortex were unequivocally pathological. Obvious atrophy and basal ganglia signal changes appeared only 9 months after the onset. Brain biopsy confirmed Creutzfeldt-Jakob disease (CJD). In rapidly progressive dementia, we recommend DWI for early diagnosis of CJD. (orig.)

  1. 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.

  2. White matter involvement in sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Caverzasi, Eduardo; Mandelli, Maria Luisa; DeArmond, Stephen J; Hess, Christopher P; Vitali, Paolo; Papinutto, Nico; Oehler, Abby; Miller, Bruce L; Lobach, Irina V; Bastianello, Stefano; Geschwind, Michael D; Henry, Roland G

    2014-12-01

    Sporadic Creutzfeldt-Jakob disease is considered primarily a disease of grey matter, although the extent of white matter involvement has not been well described. We used diffusion tensor imaging to study the white matter in sporadic Creutzfeldt-Jakob disease compared to healthy control subjects and to correlated magnetic resonance imaging findings with histopathology. Twenty-six patients with sporadic Creutzfeldt-Jakob disease and nine age- and gender-matched healthy control subjects underwent volumetric T1-weighted and diffusion tensor imaging. Six patients had post-mortem brain analysis available for assessment of neuropathological findings associated with prion disease. Parcellation of the subcortical white matter was performed on 3D T1-weighted volumes using Freesurfer. Diffusion tensor imaging maps were calculated and transformed to the 3D-T1 space; the average value for each diffusion metric was calculated in the total white matter and in regional volumes of interest. Tract-based spatial statistics analysis was also performed to investigate the deeper white matter tracts. There was a significant reduction of mean (P=0.002), axial (P=0.0003) and radial (P=0.0134) diffusivities in the total white matter in sporadic Creutzfeldt-Jakob disease. Mean diffusivity was significantly lower in most white matter volumes of interest (PCreutzfeldt-Jakob disease. Mean diffusivity reduction reflected concomitant decrease of both axial and radial diffusivity, without appreciable changes in white matter anisotropy. Tract-based spatial statistics analysis showed significant reductions of mean diffusivity within the white matter of patients with sporadic Creutzfeldt-Jakob disease, mainly in the left hemisphere, with a strong trend (P=0.06) towards reduced mean diffusivity in most of the white matter bilaterally. In contrast, by visual assessment there was no white matter abnormality either on T2-weighted or diffusion-weighted images. Widespread reduction in white matter mean

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

    Energy Technology Data Exchange (ETDEWEB)

    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

  4. Abnormal Eye Movements in Creutzfeldt-Jakob Disease

    Science.gov (United States)

    Grant, Michael P.; Cohen, Mark; Petersen, Robert B.; Halmagyi, G. Michael; McDougall, Alan; Tusa, Ronald J.; Leigh, R. John

    1993-01-01

    We report 3 patients with autopsy-proven Creutzfeldt-Jakob disease who, early in their course, developed abnormal eye movements that included periodic alternating nystagmus and slow vertical saccades. These findings suggested involvement of the cerebellar nodulus and uvula, and the brainstem reticular formation, respectively. Cerebellar ataxia was also an early manifestation and, in one patient, a frontal lobe brain biopsy was normal at a time when ocular motor and cerebellar signs were conspicuous. As the disease progressed, all saccades and quick phases of nystagmus were lost, but periodic alternating gaze deviation persisted. At autopsy, 2 of the 3 patients had pronounced involvement of the cerebellum, especially of the midline structures. Creutzfeldt-Jakob disease should be considered in patients with subacute progressive neurological disease when cognitive changes are overshadowed by ocular motor findings or ataxia.

  5. Serial MR imaging in Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Uchino, A.; Hata, H.; Ohno, M. (Kyushu Rosai Hospital, Kitakyushu (Japan). Dept. of Radiology); Yoshinaga, M.; Shiokawa, O. (Kyushu Rosai Hospital, Kitakyushu (Japan). Stroke Care Unit)

    1991-08-01

    Serial magnetic resonance (MR) imagings of two autopsied patients with Creutzfeldt-Jakob disease (CJD) are presented. Both patients showed a dramatic progression of brain atrophy. The initial MR imagings were, however, interpreted as normal except for localized mild cortical atrophy in one patient. When a normal MR image is obtained in a demented middle-aged or aged patient, CJD may still need to be ruled out: follow up MR imaging may be useful. (orig.).

  6. Serial MR imaging in Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Uchino, A.; Hata, H.; Ohno, M.; Yoshinaga, M.; Shiokawa, O.

    1991-01-01

    Serial magnetic resonance (MR) imagings of two autopsied patients with Creutzfeldt-Jakob disease (CJD) are presented. Both patients showed a dramatic progression of brain atrophy. The initial MR imagings were, however, interpreted as normal except for localized mild cortical atrophy in one patient. When a normal MR image is obtained in a demented middle-aged or aged patient, CJD may still need to be ruled out: follow up MR imaging may be useful. (orig.)

  7. Creutzfeldt-Jakob disease: a case report and differential diagnoses.

    Science.gov (United States)

    Kojima, Gotaro; Tatsuno, Brent K; Inaba, Michiko; Velligas, Stephanie; Masaki, Kamal; Liow, Kore K

    2013-04-01

    Sporadic Creutzfeldt-Jakob disease is a rare neurodegenerative disorder of unknown etiology that causes rapidly progressive dementia. This disease is uniformly fatal and most patients die within 12 months. Clinical findings include myoclonus, visual disturbances, and cerebellar and pyramidal/extrapyramidal signs in addition to rapidly progressive cognitive and functional impairment. These findings are all non-specific and it is often difficult and challenging to diagnose premortem because of low awareness and clinical suspicion. We present a 66-year-old woman with a 5-month history of rapidly progressive dementia. After a series of extensive diagnostic examinations and continuous follow-up, she was diagnosed with probable sporadic Creutzfeldt-Jakob disease based on Centers for Disease Control and Prevention (CDC) criteria, with key findings of rapidly progressive dementia, blurry vision, extrapyramidal signs (cogwheel rigidity), and abnormal hyperintensity signals on diffusion-weighted MRI. Her symptoms progressively worsened and she died 7 months after the onset. The postmortem brain autopsy demonstrated the presence of abnormal protease-resistant prion protein by Western Blot analysis. A literature review was performed on differential diagnoses that present with rapidly progressive dementia and thereby mimic sporadic Creutzfeldt-Jakob disease. These include Alzheimer's disease, dementia with Lewy Bodies, frontotemporal dementia, meningoencephalitis, corticobasal degeneration, progressive supranuclear palsy, CADASIL, and paraneoplastic encephalomyelitis.

  8. 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

  9. Detection of infectivity in blood of persons with variant and sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Douet, Jean Yves; Zafar, Saima; Perret-Liaudet, Armand; Lacroux, Caroline; Lugan, Séverine; Aron, Naima; Cassard, Herve; Ponto, Claudia; Corbière, Fabien; Torres, Juan Maria; Zerr, Inga; Andreoletti, Olivier

    2014-01-01

    We report the presence of infectivity in erythrocytes, leukocytes, and plasma of 1 person with variant Creutzfeldt-Jakob disease and in the plasma of 2 in 4 persons whose tests were positive for sporadic Creutzfeldt-Jakob disease. The measured infectivity levels were comparable to those reported in various animals with transmissible spongiform encephalopathies.

  10. 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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  11. 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.

  12. Physical properties of the Creutzfeldt-Jakob disease agent

    Energy Technology Data Exchange (ETDEWEB)

    Sklaviadis, T.K.; Manuelidis, L.; Manuelidis, E.E.

    1989-03-01

    In this report, the authors present the first physical characterization of the Creutzfeld-Jakob disease agent. Preparations with high yields of infectivity (assayed infectious units) were obtained by a novel, gentle procedure in which initially sedimenting Gp34 (prion protein) was disaggregated by a variety of criteria with no subsequent loss of infectivity. Studies with this preparation indicate that most of the Creutzfeldt-Jakob disease agent has both a viruslike size and density. In velocity sedimentation and isopycnic sucrose gradients, infectivity comigrated with nucleic acid-protein complexes of appreciable size.

  13. Creutzfeldt-Jakob disease: Magnetic resonance imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Puvaneswary, M.; Floate, D. [John Hunter Hospital, NewCastle, NSW (Australia). Departments of Medical Imaging and Neurology; Harper, C. [Royal Prince Alfred Hospital, Sydney, NSW (Australia). Department of Neuropathology

    1999-02-01

    Rapidly progressive dementia in an adult with findings of bilateral, symmetric high signal intensity on T2-weighted sequences and normal findings on T1-weighted sequences predominantly in the deep grey matter is suggestive of Creutzfeldt-Jakob disease (CJD). The peripheral cortex may be involved, as it was in the present case. The absence of subcortical periventricular white matter high signal intensity suggests that symmetric high signal intensities within the basal ganglia and cortical grey matter are more likely to be due to a degenerative process rather than due to ischaemia, infection or tumour. Copyright (1999) Blackwell Science Pty Ltd 17 refs., 6 figs.

  14. Serial computed tomography findings in Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Schlenska, G.K.; Walter, G.F.

    1989-01-01

    Serial CT investigations of 3 patients with histologically confirmed Creutzfeldt-Jakob disease revealed persisting slight brain atrophy to progressive extreme atrophy corresponding to the absolute, not the individual duration of illness. No correlation was observed between CT findings and the patients condition or electroencephalographic results. In one case with a duration of about 16 months and a terminal brain weight of 750 g a massive bilateral, later unilateral subdural hygroma appeared which probably was caused by retraction of the brain showing an enormous atrophy. (orig.)

  15. Serial computed tomography findings in Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Schlenska, G.K.; Walter, G.F.

    1989-09-01

    Serial CT investigations of 3 patients with histologically confirmed Creutzfeldt-Jakob disease revealed persisting slight brain atrophy to progressive extreme atrophy corresponding to the absolute, not the individual duration of illness. No correlation was observed between CT findings and the patients condition or electroencephalographic results. In one case with a duration of about 16 months and a terminal brain weight of 750 g a massive bilateral, later unilateral subdural hygroma appeared which probably was caused by retraction of the brain showing an enormous atrophy. (orig.).

  16. Creutzfeldt-Jakob disease: Magnetic resonance imaging findings

    International Nuclear Information System (INIS)

    Puvaneswary, M.; Floate, D.; Harper, C.

    1999-01-01

    Rapidly progressive dementia in an adult with findings of bilateral, symmetric high signal intensity on T2-weighted sequences and normal findings on T1-weighted sequences predominantly in the deep grey matter is suggestive of Creutzfeldt-Jakob disease (CJD). The peripheral cortex may be involved, as it was in the present case. The absence of subcortical periventricular white matter high signal intensity suggests that symmetric high signal intensities within the basal ganglia and cortical grey matter are more likely to be due to a degenerative process rather than due to ischaemia, infection or tumour. Copyright (1999) Blackwell Science Pty Ltd

  17. 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

  18. Physical properties of the Creutzfeldt-Jakob disease agent

    International Nuclear Information System (INIS)

    Sklaviadis, T.K.; Manuelidis, L.; Manuelidis, E.E.

    1989-01-01

    In this report, the authors present the first physical characterization of the Creutzfeld-Jakob disease agent. Preparations with high yields of infectivity (assayed infectious units) were obtained by a novel, gentle procedure in which initially sedimenting Gp34 (prion protein) was disaggregated by a variety of criteria with no subsequent loss of infectivity. Studies with this preparation indicate that most of the Creutzfeldt-Jakob disease agent has both a viruslike size and density. In velocity sedimentation and isopycnic sucrose gradients, infectivity comigrated with nucleic acid-protein complexes of appreciable size

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

    Energy Technology Data Exchange (ETDEWEB)

    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

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

    International Nuclear Information System (INIS)

    Talbott, Sabrina D.; Sattenberg, Ronald J.; Heidenreich, Jens O.; Plato, Brian M; Parker, John

    2011-01-01

    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

  1. Creutzfeldt-Jakob disease: report of four cases and review of the literature.

    Science.gov (United States)

    Atalay, Fatma Öz; Tolunay, Şahsine; Özgün, Gonca; Bekar, Ahmet; Zarifoğlu, Mehmet

    2015-01-01

    Creutzfeldt-Jakob disease is a very rare, progressive neurodegenerative disorder that is incurable and always fatal. It is one of the transmissible spongiform encephalopathies caused by prions. Multiple vacuoles in neuropil and neuronal loss in the gray matter gives the classical sponge-like appearance of brain and are responsible for the typical clinical symptoms. In this report, we present 4 cases referred to the neurology department of Uludağ University with neurological symptoms. Patients were evaluated with electroencephalogram and magnetic resonance imaging, and performed brain biopsies for further investigation. For definitive diagnosis of Creutzfeldt-Jakob disease, accumulation of prion protein in brain was detected immunohistochemically. Patients died within weeks in consequence of rapid progression of the disease. Although Creutzfeldt-Jakob disease is an infrequent disorder, when a patient presents with characteristic clinical symptoms such as rapidly progressive dementia with myoclonus, the diagnosis of Creutzfeldt-Jakob disease should be taken into consideration.

  2. Epidemiology of Creutzfeldt-Jakob Disease: Incidence. risk factors and sulVival in European studies

    NARCIS (Netherlands)

    D.P.W.M. Wientjens (Dorothee)

    1997-01-01

    textabstractCREUTZFELDT-JAKOB DISEASE (CJO) is a rare neurodegenerative disorder with a highly interesting aetiology and potentially important public health implications. l In aetiological terms, CJD is one of the human prion diseases, characterised by rapid neurodegeneration leading to

  3. 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.

  4. Peripheral Tissue Involvement in Sporadic, Iatrogenic, and Variant Creutzfeldt-Jakob Disease

    Science.gov (United States)

    Head, Mark W.; Ritchie, Diane; Smith, Nadine; McLoughlin, Victoria; Nailon, William; Samad, Sazia; Masson, Stephen; Bishop, Matthew; McCardle, Linda; Ironside, James W.

    2004-01-01

    Human prion diseases are rare fatal neurodegenerative conditions that occur as acquired, familial, or idiopathic disorders. A key event in their pathogenesis is the accumulation of an altered form of the prion protein, termed PrPSc, in the central nervous system. A novel acquired human prion disease, variant Creutzfeldt-Jakob disease, is thought to result from oral exposure to the bovine spongiform encephalopathy agent. This disease differs from other human prion diseases in its neurological, neuropathological, and biochemical phenotype. We have used immunohistochemistry and Western blot techniques to analyze the tissue distribution and biochemical properties of PrPSc in peripheral tissues in a unique series of nine cases of variant Creutzfeldt-Jakob disease. We have compared this with the distribution and biochemical forms found in all of the major subtypes of sporadic Creutzfeldt-Jakob disease and in a case of iatrogenic Creutzfeldt-Jakob disease associated with growth hormone therapy. The results show that involvement of the lymphoreticular system is a defining feature of variant Creutzfeldt-Jakob disease, but that the biochemical isoform of PrPSc found is influenced by the cell type in which it accumulates. PMID:14695328

  5. Creutzfeldt-Jakob disease surveillance in Australia: update to December 2014.

    Science.gov (United States)

    Klug, Genevieve M; Boyd, Alison; Sarros, Shannon; Stehmann, Christiane; Simpson, Marion; McLean, Catriona; Masters, Colin L; Collins, Steven J

    2016-06-30

    Nation-wide surveillance of human transmissible spongiform encephalopathies (also known as prion diseases), the most common being Creutzfeldt-Jakob disease, is performed by the Australian National Creutzfeldt-Jakob Disease Registry, based at the University of Melbourne. Prospective surveillance has been undertaken since 1993 and over this dynamic period in transmissible spongiform encephalopathy research and understanding, the unit has evolved and adapted to changes in surveillance practices and requirements concomitant with the emergence of new disease subtypes, improvements in diagnostic capabilities and the overall heightened awareness of prion diseases in the health care setting. In 2014, routine national surveillance continued and this brief report provides an update of the cumulative surveillance data collected by the Australian National Creutzfeldt-Jakob Disease Registry prospectively from 1993 to December 2014, and retrospectively to 1970.

  6. Creutzfeldt-Jakob disease surveillance in Australia: update to December 2015.

    Science.gov (United States)

    Klug, Genevieve M; Boyd, Alison; Sarros, Shannon; Stehmann, Christiane; Simpson, Marion; McLean, Catriona A; Masters, Colin L; Collins, Steven J

    2016-09-30

    Nation-wide surveillance of human transmissible spongiform encephalopathies (also known as prion diseases), the most common being Creutzfeldt-Jakob disease, is performed by the Australian National Creutzfeldt-Jakob Disease Registry, based at the University of Melbourne. Prospective surveillance has been undertaken since 1993 and over this dynamic period in transmissible spongiform encephalopathy research and understanding, the unit has evolved and adapted to changes in surveillance practices and requirements concomitant with the delineation of new disease subtypes, improvements in diagnostic capabilities and the overall heightened awareness of prion diseases in the health care setting. In 2015, routine national surveillance continued and this brief report provides an update of the cumulative surveillance data collected by the Australian National Creutzfeldt-Jakob Disease Registry prospectively from 1993 to December 2015, and retrospectively to 1970.

  7. Creutzfeldt-Jakob disease: the value of MRI

    International Nuclear Information System (INIS)

    Urbach, H.; Tschampa, H.J.; Keller, E.; Schild, H.H.; Paus, S.

    2001-01-01

    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.) [de

  8. Can Creutzfeldt-Jakob disease unravel the mysteries of Alzheimer?

    Science.gov (United States)

    Kovacs, Gabor G

    2016-09-02

    Recent studies on iatrogenic Creutzfeldt-Jakob disease (CJD) raised concerns that one of the hallmark lesions of Alzheimer disease (AD), amyloid-β (Aβ), may be transmitted from human-to-human. The neuropathology of AD-related lesions is complex. Therefore, many aspects need to be considered in deciding on this issue. Observations of recent studies can be summarized as follows: 1) The frequency of iatrogenic CJD cases with parencyhmal and vascular Aβ deposits is statistically higher than expected; 2) The morphology and distribution of Aβ deposition may show distinct features; 3) The pituitary and the dura mater themselves may serve as potential sources of Aβ seeds; 4) Cadaveric dura mater from 2 examined cases shows Aβ deposition; and 5) There is a lack of evidence that the clinical phenotype of AD appears following the application of cadaveric pituitary hormone or dura mater transplantation. These studies support the notion that neurodegenerative diseases have common features regarding propagation of disease-associated proteins as seeds. However, until further evidence emerges, prions of transmissible spongiform encephalopathies are the only neurodegenerative disease-related proteins proven to propagate clinicopathological phenotypes.

  9. Intracranial Procedures and Expected Frequency of Creutzfeldt-Jakob Disease.

    Science.gov (United States)

    Abrams, Joseph Y; Maddox, Ryan A; Schonberger, Lawrence B; Belay, Ermias D

    2016-01-01

    To assess the frequency and characteristics of intracranial procedures (ICPs) performed and the number of U.S. residents living with a history of ICP. These data are used to calculate the expected annual number of sporadic Creutzfeldt-Jakob disease (CJD) cases among U.S. residents with a history of ICP. The Nationwide Inpatient Sample provided data on the frequency and types of ICPs, and data from the National Center for Health Statistics was used to produce age-adjusted mortality rates. A model was constructed, which estimated long-term survival and sporadic CJD rates among ICP patients based on procedure type and age. There were an estimated 2,070,488 ICPs in the United States from 1998 to 2007, an average of over 200,000 per year. There were an estimated 2,023,726 U.S. residents in 2013 with a history of ICP in the previous 30 years. In 2013, there was expected to be 4.1 sporadic CJD cases (95% CI 1-8) among people with a history of ICP in the past 30 years. The considerable proportion of U.S. residents living with a history of ICP is important information for retrospective assessments of CJD or any other suspected long-term outcome of ICPs. © 2015 S. Karger AG, Basel.

  10. The contribution of different prion protein types and host polymorphisms to clinicopathological variations in Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Head, Mark W; Ironside, James W

    2012-07-01

    Creutzfeldt-Jakob disease is a fatal neurodegenerative disease that primarily affects the central nervous system. In this respect, it can be considered alongside the more frequently occurring neurodegenerative diseases, such as Alzheimer's disease. Creutzfeldt-Jakob disease is perhaps the paradigmatic protein misfolding disorder, so comparisons between the mechanisms involved in Creutzfeldt-Jakob disease and other neurodegenerative diseases associated with protein misfolding (such as the tauopathies and synucleinopathies) may also be informative. Like many of these diseases, Creutzfeldt-Jakob disease occurs sporadically or can, more rarely, be associated with mutations. However, Creutzfeldt-Jakob disease can also be acquired and is experimentally transmissible. These properties have had profound public health implications and made the disease of interest to virologists, in addition to those interested in protein misfolding disorders and neurodegeneration. The possible causes for the pronounced phenotypic variation among different forms of Creutzfeldt-Jakob disease are beginning to become understood, and these appear to depend in large measure on the genetics of the host (specifically the sequence of the prion protein gene, PRNP) and the epigenetic aspects of the agent (thought to be a misfolded and aggregated form of the PRNP gene product, termed a prion). This review will examine whether this model in its present form has sufficient complexity and subtlety to account for the clinicopathological variation evident in Creutzfeldt-Jakob disease and will outline the ways in which a more complete and informative molecular definition of human prions are currently being sought. Copyright © 2012 John Wiley & Sons, Ltd.

  11. Tau pathology in Creutzfeldt-Jakob disease revisited.

    Science.gov (United States)

    Kovacs, Gabor G; Rahimi, Jasmin; Ströbel, Thomas; Lutz, Mirjam I; Regelsberger, Günther; Streichenberger, Nathalie; Perret-Liaudet, Armand; Höftberger, Romana; Liberski, Pawel P; Budka, Herbert; Sikorska, Beata

    2017-05-01

    Creutzfeldt-Jakob disease (CJD) is a human prion disease with different etiologies. To determine the spectrum of tau pathologies in CJD, we assessed phospho-Tau (pTau) immunoreactivities in 75 sporadic CJD cases including an evaluation of the entorhinal cortex and six hippocampal subregions. Twelve cases (16%) showed only small tau-immunoreactive neuritic profiles. Fifty-two (69.3%) showed additional tau pathology in the medial temporal lobe compatible with primary age related tauopathy (PART). In 22/52 cases the lower pTau immunoreactivity load in the entorhinal cortex as compared to subiculum, dentate gyrus or CA4 region of the hippocampus was significantly different from the typical distribution of the Braak staging. A further 11 cases (14.7%) showed widespread tau pathologies compatible with features of primary tauopathies or the gray matter type of ageing-related tau astrogliopathy (ARTAG). Prominent gray matter ARTAG was also observed in two out of three additionally examined V203I genetic CJD cases. Analysis of cerebrospinal fluid revealed prominent increase of total tau protein in cases with widespread tau pathology, while pTau (T181) level was increased only in four. This correlated with immunohistochemical observations showing less pathology with anti-pTau T181 antibody when compared to anti-pTau S202/T205, T212/S214 and T231. The frequency of tau pathologies is not unusually high in sporadic CJD and does not precisely relate to PrP deposition. However, the pattern of hippocampal tau pathology often deviates from the stages of Braak. Currently applied examination of cerebrospinal fluid pTau (T181) level does not reliably reflect primary tauopathies, PART and ARTAG seen in CJD brains. © 2016 The Authors. Brain Pathology published by John Wiley & Sons Ltd on behalf of International Society of Neuropathology.

  12. Creutzfeldt-Jakob Disease: Analysis of Four Cases

    Directory of Open Access Journals (Sweden)

    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.

  13. 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

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

    NARCIS (Netherlands)

    E.A. Croes (Esther); F. Forey; G.H. Jansen; P.C. Nijssen; C.M. van Duijn (Cornelia)

    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. 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.J. 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 (Cornelia)

    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

  16. Beyond PrPres type 1/Type 2 dichotomy in Creutzfeldt-Jakob disease

    NARCIS (Netherlands)

    Uro-Coste, E.; Cassard, H.; Simon, S.; Lugan, S.; Bilheude, J.M.; Perret-Liaudet, A.; Ironside, J.E.; Haik, S.; Basset-Leobon, C.; Lacroux, C.; Peoch, K.; Streichenberger, N.; Langeveld, J.P.M.; Head, M.W.; Grassi, J.; Hauw, J.J.; Schelcher, F.; Delisle, M.B.; Andreoletti, O.

    2008-01-01

    Sporadic Creutzfeldt-Jakob disease (sCJD) cases are currently subclassified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the proteinase K (PK) digested abnormal prion protein (PrPres) identified on Western blotting (type 1 or type 2). These biochemically distinct

  17. MRI evidence of cerebellar and hippocampal involvement in Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Poon, M.A. [Dept. of Neurology, Alfred Hospital, Victoria (Australia); Stuckey, S. [Dept. of Radiology, Alfred Hospital, Victoria (Australia); Storey, E. [Van Cleef Roet Centre for Nervous Diseases, Monash Univ., Victoria (Australia)

    2001-09-01

    We report a 51-year-old woman with the Brownell-Oppenheimer (cerebellar) variant of Creutzfeldt-Jakob disease (CJD). She had the typical findings of bilateral basal ganglion changes on MRI, as well as changes in the cerebellum and hippocampus. This case adds further information to the known imaging characteristics of CJD. (orig.)

  18. MRI evidence of cerebellar and hippocampal involvement in Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Poon, M.A.; Stuckey, S.; Storey, E.

    2001-01-01

    We report a 51-year-old woman with the Brownell-Oppenheimer (cerebellar) variant of Creutzfeldt-Jakob disease (CJD). She had the typical findings of bilateral basal ganglion changes on MRI, as well as changes in the cerebellum and hippocampus. This case adds further information to the known imaging characteristics of CJD. (orig.)

  19. 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

  20. 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

  1. 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 (Cornelia); 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

  2. 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.

  3. 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.

  4. 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

  5. Neuropathological correlations with the computed tomograms in Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Nagura, Hiroshi; Tohgi, Hideo; Yamanouchi, Hiroshi (Tokyo Metropolitan Geriatric Medical Center (Japan)); Tomonaga, Masanori

    1983-03-01

    Findings of computed tomograms were correlated with pathological changes in 3 autopsied cases of Creutzfeldt-Jakob disease who died at various stages of the disease. CTs were almost normal at the periods when severe dementia, myoclonus fully developed. The brain from a patient who died at this period showed slight nerve cell loss and spongiform changes mainly in the cerebral cortex. CTs of two advanced cases showed that the atrophic processes of the brain progressed rapidly. In these cases severe nerve cell loss and status spongiosus were found in the cerebral cortex, basal nuclei and cerebellum. Serial CTs showed that atrophic processes involved first the cerebral cortex, and then the basal nuclei and cerebellum. These observations pose the problem whether the difference in the distribution of lesions observed in cases of Creutzfeldt-Jakob disease is merely due to the different stages of the disease at the time of death or due to the variety of pathologic processes in individual case.

  6. Neuropathological correlations with the computed tomograms in Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Nagura, Hiroshi; Tohgi, Hideo; Yamanouchi, Hiroshi; Tomonaga, Masanori.

    1983-01-01

    Findings of computed tomograms were correlated with pathological changes in 3 autopsied cases of Creutzfeldt-Jakob disease who died at various stages of the disease. CTs were almost normal at the periods when severe dementia, myoclonus fully developed. The brain from a patient who died at this period showed slight nerve cell loss and spongiform changes mainly in the cerebral cortex. CTs of two advanced cases showed that the atrophic processes of the brain progressed rapidly. In these cases severe nerve cell loss and status spongiosus were found in the cerebral cortex, basal nuclei and cerebellum. Serial CTs showed that atrophic processes involved first the cerebral cortex, and then the basal nuclei and cerebellum. These observations pose the problem whether the difference in the distribution of lesions observed in cases of Creutzfeldt-Jakob disease is merely due to the different stages of the disease at the time of death or due to the variety of pathologic processes in individual case. (author)

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

    OpenAIRE

    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 s...

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

    OpenAIRE

    Grau-Rivera, O.; Sánchez del Valle Díaz, Raquel; Saiz Hinajeros, Albert; Molinuevo, José L.; Bernabé, Reyes; Munteis, Elvira; Pujadas, Francesc; Salvador, Antoni; Saura, Júlia; Ugarte, Antonio; Titulaer, Maarten; Dalmau Obrador, Josep; Graus Ribas, Francesc

    2014-01-01

    IMPORTANCE 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. OBJECTIVE To determine the frequency of NSA-abs in the cerebrospinal fluid of patients with suspected CJD and in patients with pathologically confirmed (ie, definite) CJD. DESIGN, SETTING, AND PARTICIPANTS A mixed ...

  9. Anesthetic management in patients suspected of Creutzfeldt-Jakob disease -A case report-.

    Science.gov (United States)

    In, Chi Bum; Choi, Young Sil; Park, Eun Young; Chang, Dong Jin; Lee, Soo Kyung; Choi, Hyun; Moon, Hyun Soo

    2011-09-01

    Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder in which accumulation of the pathogenic prion protein induces neuronal damage and results in distinct pathologic features. This abnormal prion is an infectious protein and resistant to methods of sterilization currently being used. Therefore, management of definite, or suspected CJD patients requires additional precautions. We report our experience of a patient who had undergone brain biopsy for suspected of CJD. The patient was confirmed to have sporadic CJD.

  10. Creutzfeldt-Jakob disease and lyophilised dura mater grafts: report of two cases.

    Science.gov (United States)

    Esmonde, T; Lueck, C J; Symon, L; Duchen, L W; Will, R G

    1993-01-01

    Two further cases of Creutzfeldt-Jakob disease (CJD) in association with cadaveric dura mater grafts are described. The clinical features of all such reported cases resemble more closely those of sporadic CJD, in contrast with kuru and the cases of CJD which have arisen after therapy with human pituitary-derived growth hormone. This observation may reflect the route of inoculation of the agent. PMID:8410042

  11. Creutzfeldt-Jakob disease surveillance in Australia, update to December 2013.

    Science.gov (United States)

    Klug, Genevieve M; Boyd, Alison; Sarros, Shannon; Stehmann, Christiane; Simpson, Marion; McLean, Catriona A; Masters, Collin L; Collins, Stephen J

    2014-12-31

    Nation-wide surveillance of transmissible spongiform encephalopathies including Creutzfeldt-Jakob disease, is performed by the Australian National Creutzfeldt-Jakob Disease Registry, based at the University of Melbourne. Surveillance has been undertaken since 1993. Over this dynamic period in transmissible spongiform encephalopathy research and understanding, the unit has evolved and adapted to changes in surveillance practices and requirements, the emergence of new disease subtypes, improvements in diagnostic capabilities and the overall heightened awareness and understanding of Creutzfeldt-Jakob disease and other transmissible spongiform encephalopathies in the health care setting. In 2013, routine surveillance continued and this brief report provides an update of the surveillance data collected by the Australian National Creutzfeldt-Jakob Disease Registry prospectively from 1993 to December 2013, and retrospectively to 1970. The report highlights the recent multi-national collaborative study published that has verified the correlation between surveillance intensity and reported disease incidence. This work is copyright. You may download, display, print and reproduce the whole or part of this work in unaltered form for your own personal use or, if you are part of an organisation, for internal use within your organisation, but only if you or your organisation do not use the reproduction for any commercial purpose and retain this copyright notice and all disclaimer notices as part of that reproduction. Apart from rights to use as permitted by the Copyright Act 1968 or allowed by this copyright notice, all other rights are reserved and you are not allowed to reproduce the whole or any part of this work in any way (electronic or otherwise) without first being given the specific written permission from the Commonwealth to do so. Requests and inquiries concerning reproduction and rights are to be sent to the Online, Services and External Relations Branch, Department of

  12. 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.

  13. Extent of misclassification of death from Creutzfeldt-Jakob disease in England 1979-96: retrospective examination of clinical records

    Science.gov (United States)

    Majeed, Azeem; Lehmann, Petra; Kirby, Liz; Knight, Richard; Coleman, Michel

    2000-01-01

    Objective To investigate the extent to which deaths from Creutzfeldt-Jakob disease were misclassified during 1979-96. Design Structured review of clinical records based on predetermined criteria to determine whether death could have been due to sporadic or variant Creutzfeldt-Jakob disease. Setting 100 health authorities and 275 NHS trusts in England. Subjects 1485 people who died aged 15-44 years from selected neurological disorders in England during 1979-96. Main outcome measure Cause of death. Results The clinical records of 705 (48%) subjects were successfully traced. Tracing of clinical records was highest in subjects who died during 1990-6. There was sufficient information in the records of 640 (91%) of the 705 subjects to exclude Creutzfeldt-Jakob disease as a cause of death. In 61 (9%) subjects, there was insufficient information to reach any conclusion about the validity of the cause of death recorded on the death certificate. The clinical records of four subjects were examined further by the National Creutzfeldt-Jakob Disease Surveillance Unit; none was thought to have died from Creutzfeldt-Jakob disease. Conclusions No new cases of sporadic or variant Creutzfeldt-Jakob disease were detected in a sample of deaths most likely to have included misclassified cases. This suggests that the surveillance system is unlikely to have missed a significant number of cases among people aged 15-44 years. Hence, any rapid increase in the number of cases of variant Creutzfeldt-Jakob disease in this age group is likely to be real not artefactual. PMID:10634732

  14. 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.

  15. Diffusion-weighted MR imaging in biopsy-proven Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyo Cheol; Chang, Kee Hyun; Song In Chan; Lee, Sang Hyun; Kwon, Bae Ju; Han, Moon Hee; Kim, Sang Yun [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2001-12-01

    To compare conventional and diffusion-weighted MR imaging in terms of their depiction of the abnormalities occurring in Creutzfeldt-Jakob disease. We retrospectively analyzed the findings of conventional (T2-weighted and fluid-attenuated inversion recovery) and diffusion-weighted MR imaging in four patients with biopsy-proven Creutzfeldt-Jakob disease. The signal intensity of the lesion was classified by visual assessment as markedly high, slightly high, or isointense, relative to normal brain parenchyma. Both conventional and diffusion-weighted MR images demonstrated bilateral high signal intensity in the basal ganglia in all four patients. Cortical lesions were observed on diffusion-weighted MR images in all four, and on fluidattenuated inversion recovery MR images in one, but in no patient on T2-weighted images. Conventional MR images showed slightly high signal intensity in all lesions, while diffusion-weighted images showed markedly high signal intensity in most. Diffusion-weighted MR imaging is more sensitive than its conventional counterpart in the depiction of Creutzfeldt-Jakob disease, and permits better detection of the lesion in both the cerebral cortices and basal ganglia.

  16. Diffusion-weighted MR imaging in biopsy-proven Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Kim, Hyo Cheol; Chang, Kee Hyun; Song In Chan; Lee, Sang Hyun; Kwon, Bae Ju; Han, Moon Hee; Kim, Sang Yun

    2001-01-01

    To compare conventional and diffusion-weighted MR imaging in terms of their depiction of the abnormalities occurring in Creutzfeldt-Jakob disease. We retrospectively analyzed the findings of conventional (T2-weighted and fluid-attenuated inversion recovery) and diffusion-weighted MR imaging in four patients with biopsy-proven Creutzfeldt-Jakob disease. The signal intensity of the lesion was classified by visual assessment as markedly high, slightly high, or isointense, relative to normal brain parenchyma. Both conventional and diffusion-weighted MR images demonstrated bilateral high signal intensity in the basal ganglia in all four patients. Cortical lesions were observed on diffusion-weighted MR images in all four, and on fluidattenuated inversion recovery MR images in one, but in no patient on T2-weighted images. Conventional MR images showed slightly high signal intensity in all lesions, while diffusion-weighted images showed markedly high signal intensity in most. Diffusion-weighted MR imaging is more sensitive than its conventional counterpart in the depiction of Creutzfeldt-Jakob disease, and permits better detection of the lesion in both the cerebral cortices and basal ganglia

  17. 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.

  18. Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Bishop, Matthew T; Diack, Abigail B; Ritchie, Diane L; Ironside, James W; Will, Robert G; Manson, Jean C

    2013-04-01

    Blood transfusion has been identified as a source of human-to-human transmission of variant Creutzfeldt-Jakob disease. Three cases of variant Creutzfeldt-Jakob disease have been identified following red cell transfusions from donors who subsequently developed variant Creutzfeldt-Jakob disease and an asymptomatic red cell transfusion recipient, who did not die of variant Creutzfeldt-Jakob disease, has been identified with prion protein deposition in the spleen and a lymph node, but not the brain. This individual was heterozygous (MV) at codon 129 of the prion protein gene (PRNP), whereas all previous definite and probable cases of variant Creutzfeldt-Jakob disease have been methionine homozygotes (MM). A critical question for public health is whether the prion protein deposition reported in peripheral tissues from this MV individual correlates with infectivity. Additionally it is important to establish whether the PRNP codon 129 genotype has influenced the transmission characteristics of the infectious agent. Brain and spleen from the MV blood recipient were inoculated into murine strains that have consistently demonstrated transmission of the variant Creutzfeldt-Jakob disease agent. Mice were assessed for clinical and pathological signs of disease and transmission data were compared with other transmission studies in variant Creutzfeldt-Jakob disease, including those on the spleen and brain of the donor to the index case. Transmission of variant Creutzfeldt-Jakob disease was observed from the MV blood recipient spleen, but not from the brain, whereas there was transmission from both spleen and brain tissues from the red blood cell donor. Longer incubation times were observed for the blood donor spleen inoculum compared with the blood donor brain inoculum, suggesting lower titres of infectivity in the spleen. The distribution of vacuolar pathology and abnormal prion protein in infected mice were similar following inoculation with both donor and recipient spleen

  19. Case series of Creutzfeldt-Jakob disease in a third-level hospital in Quito.

    Science.gov (United States)

    Torres Herrán, Germaine Eleanor; Ortega Herrera, Andrés Damián; Burbano, Braulio Martinez; Serrano-Dueñas, Marcos; Ortiz Yepez, María Angélica; Barrera Madera, Raúl Alberto; Masabanda Campaña, Luis Alfredo; Baño Jiménez, Guillermo David; Santos Saltos, Denny Maritza; Correa Díaz, Edgar Patricio

    2018-04-27

    Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder that affects mammals and humans. The prevalence of this disease in the United States is 0.5 to 1 per million inhabitants. So far in Ecuador, we do not know what the prevalence or incidence is, and only one case report has been written. We present a case series of Creutzfeldt-Jakob disease in a third-level hospital in Quito. The average age of symptom onset in our patients was 58.8 years. The male to female ratio was 1:1. Two patients began with cognitive/behavioral symptoms, while 4 patients began with focal neurological signs; 1 case with ataxia, 2 with gait disorders and 1 with vertigo and headache. All of the patients had the clinical features established by the World Health Organization. In addition, the entire cohort was positive for the 14-3-3 protein in cerebrospinal fluid, and had high signal abnormalities in caudate and putamen nucleus in DWI and FLAIR IRM. Only in one case, did we reach a definitive diagnosis through a pathological study. All other cases had a probable diagnosis. In this series of cases, 6 out of 6 patients died. The average time from the onset of the symptoms to death in this cohort was 13 months. This is the first report of a series of cases of Creutzfeldt-Jakob disease in Quito. Although definitive diagnosis must be histopathological, there are ancillary tests currently available that have allowed us to obtain a diagnosis of the disease.

  20. 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

    ) 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...

  1. Topodiagnosis in Creutzfeldt-Jakob disease by HMPAO-SPECT. Topodiagnostik der Creutzfeldt-Jakobschen Krankheit durch HMPAO-SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Heye, N. (Neurologische Klinik, St. Josef Hospital, Bochum Univ. (Germany)); Farahati, J. (Klinik und Poliklinik fuer Nuklearmedizin, Essen Univ. (Gesamthochschule) (Germany)); Heinz, A. (Neurologische Klinik, St. Josef Hospital, Bochum Univ. (Germany)); Buettner, T. (Neurologische Klinik, St. Josef Hospital, Bochum Univ. (Germany)); Przuntek, H. (Neurologische Klinik, St. Josef Hospital, Bochum Univ. (Germany)); Reiners, C. (Klinik und Poliklinik fuer Nuklearmedizin, Essen Univ. (Gesamthochschule) (Germany))

    1993-02-01

    A 80-year old female presented with an early stage of Creutzfeldt-Jakob disease with clinical, neurophysiological and neuropathological findings suggesting a focal involvement of the brain. HMPAO SPECT disclosed asymmetries of regional cerebral perfusion, thus suggesting that it may be a further diagnostic instrument in this disease. (orig.)

  2. Similarities of Variant Creutzfeldt-Jakob Disease Strain in Mother and Son in Spain to UK Reference Case.

    Science.gov (United States)

    Diack, Abigail B; Boyle, Aileen; Ritchie, Diane; Plinston, Chris; Kisielewski, Dorothy; de Pedro-Cuesta, Jesús; Rábano, Alberto; Will, Robert G; Manson, Jean C

    2017-09-01

    We investigated transmission characteristics of variant Creutzfeldt-Jakob disease in a mother and son from Spain. Despite differences in patient age and disease manifestations, we found the same strain properties in these patients as in UK vCJD cases. A single strain of agent appears to be responsible for all vCJD cases to date.

  3. 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...

  4. The association of neuroleptic sensitivity in Lewy body disease with a false positive clinical diagnosis of Creutzfeldt-Jakob disease

    NARCIS (Netherlands)

    Lemstra, A. W.; Schoenmaker, N.; Rozemuller-Kwakkel, A. J. M.; van Gool, W. A.

    2006-01-01

    BACKGROUND: Dementia with Lewy bodies (DLB) and Creutzfeldt-Jakob disease (CJD) share clinical features like cognitive decline, motor disturbances en psychiatric symptoms. Overlapping symptoms may cause physicians to mistake DLB for CJD. METHODS: Clinical data of 12 patients with autopsy-confirmed

  5. Progressive fatal dementia (Creutzfeldt-Jakob disease) in a patient who received homograft tissue for tympanic membrane closure

    NARCIS (Netherlands)

    Tange, R. A.; Troost, D.; Limburg, M.

    1990-01-01

    We report the case history of a 54-year-old man who developed a fatal neurological disorder 4 years after a successful tympanoplasty with homograft pericardium. The final diagnosis of this case was Creutzfeldt-Jakob disease. This infectious spongiform encephalopathy is probably caused by a slow

  6. 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 (Cornelia)

    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

  7. Sporadic Creutzfeldt-Jakob disease in a native Puerto Rican patient.

    Science.gov (United States)

    Del Pilar-Morales, Esteban A; Cali, Ignazio; Chapas, Javier; Bertrán-Pasarell, Jorge; Puoti, Gianfranco; Gambetti, Pierluigi; Nobo, Ulises

    2015-03-01

    The diagnosis of Creutzfeldt-Jakob disease (CJD) is often a challenge for most physicians given its extremely low incidence and different clinico-pathological presentations. We report the case of a 56-year old patient native to Puerto Rico suspected of sporadic Creutzfeldt-Jakob disease (sCD). The symptoms at onset were notorious for bilateral cortical blindness followed by rapidly progressive cognitive decline, visual deficit, increased levels of CSF 14-3-3 and tau along with positive brain MRI and EEG, are highly indicative of CJD. The definite diagnosis was confirmed by the National Prion Disease Pathology Surveillance Center (NPDPSC), in Cleveland, Ohio, USA. Lack of genetic mutations in the prion protein (PrP) gene, widespread histopathological changes and the accumulation of scrapie PrP (PrPSc) in the brain confirmed the diagnosis of sCJD. The patient, admitted to our institution in 2011, represents the first detailed report of sCJD in a native Puerto Rican patient living in Puerto Rico.

  8. Mitochondrial DNA differentiates Alzheimer's disease from Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Podlesniy, Petar; Llorens, Franc; Golanska, Ewa; Sikorska, Beata; Liberski, Pawel; Zerr, Inga; Trullas, Ramon

    2016-05-01

    Low content of cell-free mitochondrial DNA (mtDNA) in cerebrospinal fluid (CSF) is a biomarker of early stage Alzheimer's disease (AD), but whether mtDNA is altered in a rapid neurodegenerative dementia such as Creutzfeldt-Jakob disease is unknown. CSF mtDNA was measured using digital polymerase chain reaction (dPCR) in two independent cohorts comprising a total of 112 patients diagnosed with sporadic Creutzfeldt-Jakob disease (sCJD), probable AD, or non-Alzheimer's type dementia. Patients with AD exhibit low mtDNA content in CSF compared with patients diagnosed with sCJD or with non-Alzheimer's type dementias. The CSF concentration of mtDNA does not correlate with Aβ, t-tau, p-tau, and 14-3-3 protein levels in CSF. Low-CSF mtDNA is not a consequence of brain damage and allows the differential diagnosis of AD from sCJD and other dementias. These results support the hypothesis that mtDNA in CSF is a pathophysiological biomarker of AD. Copyright © 2015 Alzheimer's Association. Published by Elsevier Inc. All rights reserved.

  9. Applicability of long-term electroencephalography in pre-mortem diagnosis of Creutzfeldt-Jakob disease: A case report.

    Science.gov (United States)

    Attaripour Isfahani, Sanaz; Dougherty, Michelle; Gliebus, Gediminas Peter

    2017-01-01

    Creutzfeldt-Jakob disease accounts for more than 90% of all sporadic prion disease cases. The molecular MM2 genotype has been divided into cortical and thalamic subtypes based on structures involved and is characterized clinically by progressive dementia without ataxia or typical electroencephalography changes. Proposed diagnostic criteria for MM2 cortical type sporadic Creutzfeldt-Jakob disease include progressive dementia, cortical hyper-intensity on diffusion-weighted magnetic resonance imaging, increased cerebrospinal fluid 14-3-3 protein level, and the exclusion of other types of dementia. The presence of periodic discharges on electroencephalography in MM2 cortical type were reported in 42% of the cases. We are reporting a case of sporadic Creutzfeldt-Jakob disease cortical MM2-type presenting with rapid cognitive decline, who survived 8 months since symptom onset. Brain imaging, cerebrospinal fluid analysis, and long-term electroencephalography monitoring were obtained and diagnosis was confirmed by autopsy. Short-term electroencephalography recording, performed 5 months after symptom onset, demonstrated diffuse background slowing without epileptiform activity. Long-term video electroencephalography monitoring demonstrated generalized slowing, maximum in bilateral frontal areas, which intermittently would become rhythmic (1-2 Hz) without hemispheric predominance. If the findings do not clearly meet the proposed clinical criteria for sporadic Creutzfeldt-Jakob disease, the use of long-term electroencephalography could increase the sensitivity. We question whether the lack of the characteristic findings on electroencephalography in some cases could be due to insufficient time of recording. Application of long-term electroencephalography monitoring increases the sensitivity of electroencephalography and the certainty of pre-mortem diagnosis of sporadic Creutzfeldt-Jakob disease.

  10. Cerebrospinal Fluid Biomarkers in the Diagnosis of Creutzfeldt-Jakob Disease in Slovak Patients: over 10-Year Period Review.

    Science.gov (United States)

    Koscova, Silvia; Zakova Slivarichova, Dana; Tomeckova, Ivana; Melicherova, Katarina; Stelzer, Martin; Janakova, Alzbeta; Kosorinova, Dana; Belay, Girma; Mitrova, Eva

    2017-10-01

    Creutzfeldt-Jakob disease is a rare, but rapidly progressive, up to now untreatable and fatal neurodegenerative disorder. Clinical diagnosis of Creutzfeldt-Jakob disease (CJD) is difficult; however, it can be facilitated by suitable biomarkers. Aim of the present study is to compare levels of cerebrospinal fluid biomarkers (total tau protein, phosphorylated-tau protein, protein 14-3-3 and amyloid beta) in Slovak population of CJD suspect cases, retrospectively in over a 10-year period. One thousand three hundred sixty-four CSF samples from patients with suspect CJD, forming a homogenous group in terms of geographical as well as of equal transport conditions, storage and laboratory processing, were analysed. Definite diagnosis of Creutzfeldt-Jakob disease was confirmed in 101 patients with genetic form, and 60 patients with its sporadic form of the disease. Specificity of protein 14-3-3 and total tau in both forms CJD was similar (87 % for P14-3-3/85 % for total tau), sensitivity to P 14-3-3 and total tau was higher in sporadic Creutzfeldt-Jakob disease (sCJD) (90/95 %) than in genetic Creutzfeldt-Jakob disease (gCJD) (89/74 %). As expected, the total tau levels were significantly higher in CJD patients than in controls, but there was also significant difference between gCJD and sCJD (levels in gCJD were lower; p = 0.003). There was no significant difference in p-tau and Aβ 1-42 levels neither between both CJD forms nor between CJD patients and control group.

  11. Prion Strain Characterization of a Novel Subtype of Creutzfeldt-Jakob Disease.

    Science.gov (United States)

    Galeno, Roberta; Di Bari, Michele Angelo; Nonno, Romolo; Cardone, Franco; Sbriccoli, Marco; Graziano, Silvia; Ingrosso, Loredana; Fiorini, Michele; Valanzano, Angelina; Pasini, Giulia; Poleggi, Anna; Vinci, Ramona; Ladogana, Anna; Puopolo, Maria; Monaco, Salvatore; Agrimi, Umberto; Zanusso, Gianluigi; Pocchiari, Maurizio

    2017-06-01

    In 2007, we reported a patient with an atypical form of Creutzfeldt-Jakob disease (CJD) heterozygous for methionine-valine (MV) at codon 129 who showed a novel pathological prion protein (PrP TSE ) conformation with an atypical glycoform (AG) profile and intraneuronal PrP deposition. In the present study, we further characterize the conformational properties of this pathological prion protein (PrP TSE MV AG ), showing that PrP TSE MV AG is composed of multiple conformers with biochemical properties distinct from those of PrP TSE type 1 and type 2 of MV sporadic CJD (sCJD). Experimental transmission of CJD-MV AG to bank voles and gene-targeted transgenic mice carrying the human prion protein gene (TgHu mice) showed unique transmission rates, survival times, neuropathological changes, PrP TSE deposition patterns, and PrP TSE glycotypes that are distinct from those of sCJD-MV1 and sCJD-MV2. These biochemical and experimental data suggest the presence of a novel prion strain in CJD-MV AG IMPORTANCE Sporadic Creutzfeldt-Jakob disease is caused by the misfolding of the cellular prion protein, which assumes two different major conformations (type 1 and type 2) and, together with the methionine/valine polymorphic codon 129 of the prion protein gene, contribute to the occurrence of distinct clinical-pathological phenotypes. Inoculation in laboratory rodents of brain tissues from the six possible combinations of pathological prion protein types with codon 129 genotypes results in the identification of 3 or 4 strains of prions. We report on the identification of a novel strain of Creutzfeldt-Jakob disease isolated from a patient who carried an abnormally glycosylated pathological prion protein. This novel strain has unique biochemical characteristics, does not transmit to humanized transgenic mice, and shows exclusive transmission properties in bank voles. The identification of a novel human prion strain improves our understanding of the pathogenesis of the disease and of

  12. 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

  13. Related or not? Development of spontaneous Creutzfeldt-Jakob disease in a patient with chronic, well-controlled HIV: A case report and review of the literature.

    Science.gov (United States)

    Babi, M-Alain; Kraft, Bryan D; Sengupta, Sweta; Peterson, Haley; Orgel, Ryan; Wegermann, Zachary; Lugogo, Njira L; Luedke, Matthew W

    2016-01-01

    We report a novel case of a rare disease: spontaneous Creutzfeldt-Jakob disease in a patient with well-controlled HIV. We explore the relationship between spontaneous Creutzfeldt-Jakob disease and HIV. A 66-year-old man with long-standing, well-controlled HIV infection presented with 3 months of progressive, subacute neurocognitive decline. His symptoms included conceptual apraxia, apathy, memory impairment, and gait disturbance, and were initially attributed to depressive "pseudo-dementia." Unfortunately, the patient's symptoms rapidly progressed and he ultimately succumbed to his illness. Autopsy confirmed the clinical diagnosis of spontaneous Creutzfeldt-Jakob disease. This case highlights spontaneous Creutzfeldt-Jakob disease as a rare terminal illness in the setting of well-controlled chronic HIV. To our knowledge, this is the first report of a patient with chronic and previously well-controlled HIV infection dying from a prion disease. Despite the very different epidemiology and pathophysiology of HIV and spontaneous Creutzfeldt-Jakob disease, this case does raise questions of whether certain host genetic factors could predispose to both conditions, albeit currently, there is no clear causal link between HIV and spontaneous Creutzfeldt-Jakob disease.

  14. Atypical presentation of probable Creutzfeldt-Jakob disease associated with anti-Zic4 antibody: Literature review of neuronal antibodies in Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Salazar, Richard

    2018-05-01

    Sporadic Creutzfeldt-Jakob disease is a prion disease characterized by rapidly progressive dementia, ataxia and myoclonus. Atypical phenotype masquerading as stroke, movement disorders or autoimmune encephalitis have been described. Here, I report a probable case of sCJD with an atypical presentation associated with anti-Zic4 antibody and review the literature of neuronal antibodies in CJD. A 70 year-old gentleman is admitted with a 2-month history of recurrent stroke-like symptoms associated with behavioral disturbances, gait ataxia and rapidly progressive dementia. His initial examination demonstrated akinetic mutism, diffuse rigidity, dysautononia, and Cheyne-Stokes respiration. Over the following weeks his condition progressed to profound coma. A comprehensive infectious, metabolic, inflammatory and autoimmune work-up yielded negative results. Empiric immunosuppressive therapy ensued. He expired three months after symptoms onset. Autopsy was not performed. After his demise, prion tests came back abnormal for elevated 14-3-3 protein, total tau and positive RTQuIC. Later on, anti-Zic4 antibodies were found in serum. This case underscores the importance of a high index of suspicion for CJD even in case of atypical features or the concurrence of neuronal antibodies. Further larger prospective studies on the prevalence of these neuronal antibodies in CJD and the contribution of these autoantibodies to disease pathophysiology are necessary. Copyright © 2018 Elsevier B.V. All rights reserved.

  15. Cerebrospinal fluid biomarker supported diagnosis of Creutzfeldt-Jakob disease and rapid dementias: a longitudinal multicentre study over 10 years.

    Science.gov (United States)

    Stoeck, Katharina; Sanchez-Juan, Pascual; Gawinecka, Joanna; Green, Alison; Ladogana, Anna; Pocchiari, Maurizio; Sanchez-Valle, Raquel; Mitrova, Eva; Sklaviadis, Theodor; Kulczycki, Jerzy; Slivarichova, Dana; Saiz, Albert; Calero, Miguel; Knight, Richard; Aguzzi, Adriano; Laplanche, Jean-Louis; Peoc'h, Katell; Schelzke, Gabi; Karch, Andre; van Duijn, Cornelia M; Zerr, Inga

    2012-10-01

    To date, cerebrospinal fluid analysis, particularly protein 14-3-3 testing, presents an important approach in the identification of Creutzfeldt-Jakob disease cases. However, one special point of criticism of 14-3-3 testing is the specificity in the differential diagnosis of rapid dementia. The constant observation of increased cerebrospinal fluid referrals in the national surveillance centres over the last years raises the concern of declining specificity due to higher number of cerebrospinal fluid tests performed in various neurological conditions. Within the framework of a European Community supported longitudinal multicentre study ('cerebrospinal fluid markers') we analysed the spectrum of rapid progressive dementia diagnoses, their potential influence on 14-3-3 specificity as well as results of other dementia markers (tau, phosphorylated tau and amyloid-β(1-42)) and evaluated the specificity of 14-3-3 in Creutzfeldt-Jakob disease diagnosis for the years 1998-2008. A total of 29 022 cerebrospinal fluid samples were analysed for 14-3-3 protein and other cerebrospinal fluid dementia markers in patients with rapid dementia and suspected Creutzfeldt-Jakob disease in the participating centres. In 10 731 patients a definite diagnosis could be obtained. Protein 14-3-3 specificity was analysed for Creutzfeldt-Jakob disease with respect to increasing cerebrospinal fluid tests per year and spectrum of differential diagnosis. Ring trials were performed to ensure the comparability between centres during the reported time period. Protein 14-3-3 test specificity remained high and stable in the diagnosis of Creutzfeldt-Jakob disease during the observed time period across centres (total specificity 92%; when compared with patients with definite diagnoses only: specificity 90%). However, test specificity varied with respect to differential diagnosis. A high 14-3-3 specificity was obtained in differentiation to other neurodegenerative diseases (95-97%) and non

  16. 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.

  17. 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.

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

    Science.gov (United States)

    Caboclo, Luís Otávio Sales Ferreira; Huang, Nancy; Lepski, Guilherme Alves; Livramento, José Antônio; Buchpiguel, Carlos Alberto; Porto, Cláudia Sellitto; Nitrini, Ricardo

    2002-06-01

    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.

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

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    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. 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.

  1. Forma de heidenhain da doença de creutzfeldt-jakob: relato de um caso

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    Luciano Ribeiro Pinto Junior

    1986-03-01

    Full Text Available Registro de um caso da doença de Creutzfeldt-Jakob, forma de Heidenhain, que se caracteriza por comprometimento visual importante. As manifestações iniciais consistiram de distúrbios visuais. A paciente evoluiu para franca demência, ao lado de manifestações pirâmido-extrapiramidais. Além dos dados clínicos o EEG mostrou atividade periódica característica da doença. O estudo anátomo-patológico confirmou o diagnóstico clínico mostrando alteração esponjosa, degeneração neuronal e astrogliose. Além do quadro clínico, eletrencefalográfico e anátomo-patológico são discutidos os aspectos etiopatogênicos da doença.

  2. Long-term preclinical magnetic resonance imaging alterations in sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Zanusso, Gianluigi; Camporese, Giulia; Ferrari, Sergio; Santelli, Luca; Bongianni, Matilde; Fiorini, Michele; Monaco, Salvatore; Manara, Renzo; Cagnin, Annachiara

    2016-10-01

    An asymptomatic 74-year-old woman, on follow-up for a carotid body tumor, showed magnetic resonance imaging (MRI) focal restricted diffusion confined to the left temporal and occipital cortices. Thirteen months later, diffusion-weighted images revealed a bilateral cortical ribbon sign involving all lobes. After 1 month, the patient developed gait instability and cognitive decline rapidly evolving to severe dementia and death within 3 months. Prion protein gene sequence, molecular, and neuropathological studies confirmed the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 subtype. Here we show the kinetics of MRI changes and prion spreading in preclinical sCJD MM1. Ann Neurol 2016;80:629-632. © 2016 American Neurological Association.

  3. 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.

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

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    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)

  5. MRI in sporadic Creutzfeldt-Jakob disease: Correlation with clinical and neuropathological data

    Energy Technology Data Exchange (ETDEWEB)

    Urbach, H.; Solymosi, L. [Department of Neuroradiology, University of Wuerzburg (Germany); Klisch, J.; Brechtelsbauer, D. [Department of Neuroradiology, University of Bonn, Bonn (Germany); Wolf, H.K. [Department of Neuropathology, University of Bonn, Bonn (Germany); Gass, S. [Department of Neurology, University of Bonn, Bonn (Germany)

    1998-02-01

    To ascertain whether increased grey matter signal intensity on T2-weighted images in patients with sporadic Creutzfeldt-Jakob disease (CJD) corresponds to the stage and severity of this disease, we correlated MRI findings in four of our own and previously reported patients with sporadic CJD with the clinical variants, neuropathological changes at autopsy, duration of the disease and survival time after MRI examination. Of 15 patients with the extrapyramidal type of CJD, 10 showed increased signal in the basal ganglia on T2-weighted images. One of seven patients with the Heidenhain variant had increased signal in the occipital cortex. Patients without increased grey matter signal intensity had a longer overall duration of CJD (P = 0.035). Although the interval between onset of neurological symptoms and MRI was not different, patients without increased grey matter signal also survived longer after MRI examination (P = 0.022). (orig.) With 5 figs., 2 tabs., 23 refs.

  6. 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.

  7. Association between the PRNP 1368 polymorphism and the occurrence of sporadic Creutzfeldt-Jakob disease

    Science.gov (United States)

    Bratosiewicz-Wąsik, Jolanta; Smoleń-Dzirba, Joanna; Rozemuller, Annemieke J.; Jansen, Casper; Spliet, Wim; Jansen, Gerard H.; Wąsik, Tomasz J.; Liberski, Paweł P.

    2012-01-01

    Creutzfeldt-Jakob disease (CJD) is a rare transmissible neurodegenerative disorder. The etiology of sporadic form of CJD remains unsolved. In addition to the codon 129 polymorphism, polymorphisms in the non-coding region of PRNP are considered as important factors in sCJD development. To assess a possible association between PRNP 1368 SNP and sCJD, we compared the genotype, allele and haplotype frequencies of the 1368 SNP among 46 sCJD patients of Dutch origin with the respective frequencies in healthy controls. We detected a significant association between sCJD and 1368T/T genotype. A significant difference was also observed in 1368 alleles’ distribution. In the haplotype analysis, haplotype 1368C-129G was associated with decreased risk of sCJD in Dutch population. Our findings support the hypothesis that genetic variations in the regulatory region of the PRNP gene may influence the pathogenesis of sCJD. PMID:22895088

  8. MRI in sporadic Creutzfeldt-Jakob disease: Correlation with clinical and neuropathological data

    International Nuclear Information System (INIS)

    Urbach, H.; Solymosi, L.; Klisch, J.; Brechtelsbauer, D.; Wolf, H.K.; Gass, S.

    1998-01-01

    To ascertain whether increased grey matter signal intensity on T2-weighted images in patients with sporadic Creutzfeldt-Jakob disease (CJD) corresponds to the stage and severity of this disease, we correlated MRI findings in four of our own and previously reported patients with sporadic CJD with the clinical variants, neuropathological changes at autopsy, duration of the disease and survival time after MRI examination. Of 15 patients with the extrapyramidal type of CJD, 10 showed increased signal in the basal ganglia on T2-weighted images. One of seven patients with the Heidenhain variant had increased signal in the occipital cortex. Patients without increased grey matter signal intensity had a longer overall duration of CJD (P = 0.035). Although the interval between onset of neurological symptoms and MRI was not different, patients without increased grey matter signal also survived longer after MRI examination (P = 0.022). (orig.)

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

    International Nuclear Information System (INIS)

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

    2003-01-01

    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

  10. Early-Onset Creutzfeldt-Jakob Disease Mimicking Immune-Mediated Encephalitis

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    Wietse A. Wiels

    2018-04-01

    Full Text Available ObjectivesThe objective of this study is to explore the clinical, radiological, and pathological manifestations of a rare subtype of prion disease and their implication for differential diagnosis in case of an early onset neuropsychiatric deterioration.MethodsWe discuss a patients’ clinical history, as well as the string of investigations and symptomatological evolution that finally led to a pathological diagnosis.ResultsOur patient had the extremely rare VV1 type sporadic Creutzfeldt-Jakob disease (sCJD. We explain the differential diagnosis of progressive encephalomyelitis with rigidity and myoclonus and its implications for treatment.ConclusionsCJD, especially the VV1 subtype, can present at an early age with an insidious psychiatric onset. Classical findings of prion disease—14-3-3 protein, PSWC on electroencephalography, and magnetic resonance imaging patterns—are not always present. The presence of neural autoantibodies does not always implicate pathogenicity in the presence of other neurological/neurodegenerative conditions.

  11. MRI manifestation for the diagnosis of sporadic Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Xue Yonggang; Qi Ji; Xia Shuang

    2007-01-01

    Objective: To study the MRI features of sporadic Creutzfeldt-Jakob disease (sCJD). Methods: Three patients with clinically diagnosed sCJD underwent MR study, including SE T 1 WI, FSE T 2 WI, and DWI sequences. The MR imaging features were analyzed. Results: The lesions were not definite either in SE T 1 WI or in FSE T 2 WI, but were prominent in DWI. Abnormal hyperintensive signal appeared in the cerebral cortex, with the frontal, parietal, and occipital lobes being the mostly involved region. The subcortical white matter was normal. The bilateral caudate nuclei and thalami could also be involved. The abnormal signal could be either symmetrical or asymmetrical. There was diffuse atrophy of the brain parenchyma in the late phase of disease, especially in the cortex. Conclusion: With the application of MR study, especially the DWI, combined with its characteristic clinical manifestation, the diagnosis of sCJD can be made definitely. (authors)

  12. 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.

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

    International Nuclear Information System (INIS)

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

    2001-01-01

    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)

  14. 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

    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. Copyright © 2017 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

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

    Science.gov (United States)

    Takatsuki, Hanae; Fuse, Takayuki; Nakagaki, Takehiro; Mori, Tsuyoshi; Mihara, Ban; Takao, Masaki; Iwasaki, Yasushi; Yoshida, Mari; Murayama, Shigeo; Atarashi, Ryuichiro; Nishida, Noriyuki; Satoh, Katsuya

    2016-10-01

    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 10 6 /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 10 6 /g SD50 did not exist the infectivity. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    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.

  17. Cerebrospinal fluid tau levels are a marker for molecular subtype in sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Karch, André; Hermann, Peter; Ponto, Claudia; Schmitz, Matthias; Arora, Amandeep; Zafar, Saima; Llorens, Franc; Müller-Heine, Annika; Zerr, Inga

    2015-05-01

    The molecular subtype of sporadic Creutzfeldt-Jakob disease (sCJD) is an important prognostic marker for patient survival. However, subtype determination is not possible during lifetime. Because the rate of disease progression is associated with the molecular subtype, this study aimed at investigating if total tau, a marker of neuronal death, allows premortem diagnosis of molecular subtype when codon 129 genotype is known. Two hundred ninety-six sCJD patients were tested for their cerebrospinal fluid total tau level at the time of diagnosis and were investigated for their sCJD subtype postmortem. There was a significant association between tau levels and the prion protein type in patients with codon 129 MM (p disease. Copyright © 2015 Elsevier Inc. All rights reserved.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  19. Recent US Case of Variant Creutzfeldt-Jakob Disease-Global Implications.

    Science.gov (United States)

    Maheshwari, Atul; Fischer, Michael; Gambetti, Pierluigi; Parker, Alicia; Ram, Aarthi; Soto, Claudio; Concha-Marambio, Luis; Cohen, Yvonne; Belay, Ermias D; Maddox, Ryan A; Mead, Simon; Goodman, Clay; Kass, Joseph S; Schonberger, Lawrence B; Hussein, Haitham M

    2015-05-01

    Variant Creutzfeldt-Jakob disease (vCJD) is a rare, fatal prion disease resulting from transmission to humans of the infectious agent of bovine spongiform encephalopathy. We describe the clinical presentation of a recent case of vCJD in the United States and provide an update on diagnostic testing. The location of this patient's exposure is less clear than those in the 3 previously reported US cases, but strong evidence indicates that exposure to contaminated beef occurred outside the United States more than a decade before illness onset. This case exemplifies the persistent risk for vCJD acquired in unsuspected geographic locations and highlights the need for continued global surveillance and awareness to prevent further dissemination of vCJD.

  20. 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.

  1. New MRI findings in Creutzfeldt-Jakob disease: high signal in the globus pallidus on T 1-weighted images

    International Nuclear Information System (INIS)

    Priester, J.A. de; Wilmink, J.T.; Jansen, G.H.; Kruijk, J.R. de

    1999-01-01

    We report a 49-year-old woman with Creutzfeldt-Jakob disease (CJD). In addition to typical high-signal lesions on proton-density and T 2-weighted images there was high signal in the globus pallidus bilaterally on T 1-weighted images. The latter feature has not been described previously and probably due to deposition of prion protein, as found at autopsy. (orig.)

  2. New MRI findings in Creutzfeldt-Jakob disease: high signal in the globus pallidus on T 1-weighted images

    Energy Technology Data Exchange (ETDEWEB)

    Priester, J.A. de; Wilmink, J.T. [Dept. of Radiology, University Hospital Maastricht (Netherlands); Jansen, G.H. [Department of Neuropathology, University Hospital Utrecht (Netherlands); Kruijk, J.R. de [Department of Neurology, University Hospital Maastricht (Netherlands)

    1999-04-01

    We report a 49-year-old woman with Creutzfeldt-Jakob disease (CJD). In addition to typical high-signal lesions on proton-density and T 2-weighted images there was high signal in the globus pallidus bilaterally on T 1-weighted images. The latter feature has not been described previously and probably due to deposition of prion protein, as found at autopsy. (orig.) With 3 figs., 11 refs.

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

    OpenAIRE

    Gattás, Vera Lúcia; Lima Neto, Antonio Silva; Dimech, George Santiago; Mancini, Denise; Cantarino, Ligia Maria; Marins, José Ricardo Pio; Luna, Expedito José Albuquerque

    2007-01-01

    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 wor...

  4. Short TE quantitative proton magnetic resonance spectroscopy in variant Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Cordery, R.J.; Godbolt, A.; Rossor, M.N.; MacManus, D.; Waldman, A.D.

    2006-01-01

    Variant Creutzfeldt-Jakob disease (vCJD) is a fatal neurodegerative disorder. Clinical diagnosis is difficult in the early stages as the disease often presents with non-specific psychiatric and neurological symptoms. To investigate the diagnostic potential of quantitative short TE in vivo MRS, and the nature and anatomical distribution of biochemical abnormalities in vCJD, localised single-voxel spectra (TE/TR 30 ms/2,000 ms) were acquired from three brain regions: thalami, caudate nuclei and frontal white matter. Metabolite concentrations and ratios from three patients with definite or probable vCJD were compared with eight normal age-matched controls. Abnormal signal on T2-weighted MRI was apparent in the pulvinar region in all vCJD patients; this region also showed greatly increased myo-inositol [MI] (mean 2.5-fold, P=0.01) and decreased N-acetyl-aspartate (NAA; mean 2-fold, P=0.01). Two patients also showed increased [MI] (z=17, 11; one with decreased NAA, z=-12) in normal-appearing caudate nuclei. The magnitude of metabolite abnormalities in the thalami in moderately advanced vCJD suggests a potential role in earlier diagnosis. Short TE protocols allow the measurement of MI, which adds discriminant power to the MRS examination. (orig.)

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

  9. Combined Creutzfeldt-Jakob/ Alzheimer's Disease Cases are Important in Search for Microbes in Alzheimer's Disease.

    Science.gov (United States)

    Bastian, Frank O

    2017-01-01

    The question whether Alzheimer's disease is infectious as brought up in the recent editorial published in the Journal of Alzheimer's Disease is complicated by the controversy whether the causal agent is a microbe or a misfolded host protein (amyloid). The replicating amyloid (prion) theory, based upon data from studies of Creutzfeldt-Jakob disease (CJD) and other transmissible spongiform encephalopathies (TSEs), has been challenged since the prion can be separated from TSE infectivity, and spiroplasma, a wall-less bacterium, has been shown to be involved in the pathogenesis of CJD. Further support for a microbial cause for AD comes from occurrence of mixed CJD/AD cases involving up to 15% of AD brains submitted to brain banks. The association of CJD with AD suggests a common etiology rather than simply being a medical curiosity. A co-infection with the transmissible agent of CJD, which we propose to be a Spiroplasma sp., would explain the diversity of bacteria shown to be associated with cases of AD.

  10. Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties

    Science.gov (United States)

    Bishop, Matthew T.; Will, Robert G.; Manson, Jean C.

    2010-01-01

    The biological determinants of the phenotypic variation in sporadic Creutzfeldt-Jakob disease (sCJD) are unknown. To categorize sCJD cases, the prion protein (PrP) codon 129 genotype and the biochemical characteristics of the disease-associated form of PrP (PrPSc) can be combined to form six subgroups (MM1, MM2, MV1, MV2, VV1, and VV2). This classification largely correlates with the known variation in the clinical and pathological features of sCJD, with the MM1 and MV1 cases representing the “classic” phenotype of sCJD. To address how this classification relates to different strains of sCJD we have inoculated each subgroup of sCJD to a panel of mice expressing different forms of the human PRNP gene (129MM, 129VV, and 129MV). We have established that all subtypes are transmissible to at least one genotype of mouse, and both agent and host factors determine transmission efficiency and the form of PrPSc deposited in the brain. Moreover, we have identified four distinct strains of sCJD using our in vivo strain typing panel. PMID:20547859

  11. 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

  12. 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

    2016-11-14

    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 (PrP Sc ) 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.

  13. Psychiatric symptoms in patients with sporadic Creutzfeldt-Jakob disease in Germany.

    Science.gov (United States)

    Krasnianski, Anna; Bohling, Geeske T; Harden, Markus; Zerr, Inga

    2015-09-01

    Psychiatric symptoms in sporadic Creutzfeldt-Jakob disease (sCJD) are still not sufficiently evaluated. To describe psychiatric symptoms in sCJD with respect to molecular subtype. Patients in this retrospective study were classified according to established diagnostic criteria. 248 sCJD patients with known molecular subtype were recruited from January 1993 to December 2004 and investigated. Psychiatric symptoms were defined according to Möller and colleagues and the AMDP system (Study Group for Methods and Documentation in Psychiatry) and were collected by direct examination by study physicians or extracted from medical documentation. Our data were compared with published data on variant CJD (vCJD). Psychiatric symptoms were common in sCJD patients (90%) and mostly found already at the disease onset (agitation in 64% of the patients, hallucinations in 45%, anxiety in 50%, depression in 37%). All psychiatric symptoms but illusions were found early in the disease course. Psychiatric symptoms in sCJD were less frequent than in vCJD. We provide the first detailed analysis of psychiatric symptoms in a large group of patients with sCJD with respect to differences concerning frequency and time point of occurrence of psychiatric symptoms between molecular subtypes. These data suggest that psychiatric symptoms occurring early in the disease course are common not only in vCJD but also in other CJD types. © Copyright 2015 Physicians Postgraduate Press, Inc.

  14. Towards an age-dependent transmission model of acquired and sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    de Pedro-Cuesta, Jesús; Mahillo-Fernandez, Ignacio; Calero, Miguel; Rábano, Alberto; Cruz, Mabel; Siden, Åke; Martínez-Martín, Pablo; Laursen, Henning; Ruiz-Tovar, María; Mølbak, Kåre

    2014-01-01

    Sporadic Creutzfeldt-Jakob disease (sCJD) might be transmitted by surgery. The purpose of this study was to investigate potential susceptibility to sCJD from surgery at juvenile age and in early adulthood. From Danish and Swedish national registries we identified 167 definite and probable sCJD cases with onset from 1987 through 2003, and 835 age-, sex- and residence-matched controls along with their surgical histories. Main, anatomically or etiologically classified surgical procedures followed by a ≥20-year lag were analyzed using logistic regression, and stratified by age at first-registered surgical discharge. The risk of having a diagnosis of CJD depended strongly on age at first surgery with odds ratio (OR) of 12.80 (95% CI 2.56-64.0) in patients <30 years, 3.04 (95% 1.26-7.33) in 30-39 years, and 1.75 (95% CI 0.89-3.45) in ≥40 years, for anatomically classified surgical procedures. Similar figures were obtained for etiologically classified surgical procedures. Risk of surgical-acquired sCJD depends on age at exposure; this pattern is similar to age-specific profiles reported for CJD accidentally transmitted by human pituitary-derived growth hormone and susceptibility curves for variant CJD estimated after adjustment for dietary exposure to bovine spongiform encephalopathy. There might be an age-at-exposure-related susceptibility to acquire all CJD forms, including sCJD from routine surgery.

  15. Serum uric acid and lipid profiles in sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Chen, Shuai; He, Shuang; Shang, Jun-Kui; Ma, Ming-Ming; Xu, Chang-Shui; Shi, Xiao-Hong; Zhang, Jie-Wen

    2016-02-01

    Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and fatal neurodegenerative disease affecting the central nervous system. Brain lipid homeostasis and oxidative stress seem to play an important role in the disease pathogenesis. But little was known whether serum lipids and uric acid (a natural antioxidant) levels changed in patients with prion disease. Here we retrospectively reviewed and compared the serum lipids and uric acid levels of 19 probable sporadic CJD patients and 26 healthy control subjects. We found that the serum uric acid levels in sporadic CJD patients were significantly lower than that in controls (P=0.01). Serum triglycerides, cholesterol, low-density lipoprotein (LDL), high-density lipoprotein (HDL), and apolipoprotein A1 (ApoA1) were similar in sporadic CJD patients and controls. However, LDL/HDL ratio was lower in sporadic CJD patients (P=0.003). The low serum uric acid and LDL/HDL ratio levels in sporadic CJD indicate that dysfunction in the lipid homeostasis and oxidative stress is associated with sporadic prion disease. Copyright © 2015 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.

  16. The Prion Protein Preference of Sporadic Creutzfeldt-Jakob Disease Subtypes*

    Science.gov (United States)

    Klemm, Helen M. J.; Welton, Jeremy M.; Masters, Colin L.; Klug, Genevieve M.; Boyd, Alison; Hill, Andrew F.; Collins, Steven J.; Lawson, Victoria A.

    2012-01-01

    Sporadic Creutzfeldt-Jakob disease (CJD) is the most prevalent manifestation of the transmissible spongiform encephalopathies or prion diseases affecting humans. The disease encompasses a spectrum of clinical phenotypes that have been correlated with molecular subtypes that are characterized by the molecular mass of the protease-resistant fragment of the disease-related conformation of the prion protein and a polymorphism at codon 129 of the gene encoding the prion protein. A cell-free assay of prion protein misfolding was used to investigate the ability of these sporadic CJD molecular subtypes to propagate using brain-derived sources of the cellular prion protein (PrPC). This study confirmed the presence of three distinct sporadic CJD molecular subtypes with PrPC substrate requirements that reflected their codon 129 associations in vivo. However, the ability of a sporadic CJD molecular subtype to use a specific PrPC substrate was not determined solely by codon 129 as the efficiency of prion propagation was also influenced by the composition of the brain tissue from which the PrPC substrate was sourced, thus indicating that nuances in PrPC or additional factors may determine sporadic CJD subtype. The results of this study will aid in the design of diagnostic assays that can detect prion disease across the diversity of sporadic CJD subtypes. PMID:22930754

  17. The French surveillance network of Creutzfeldt-Jakob disease. Epidemiological data in France and worldwide.

    Science.gov (United States)

    Brandel, J-P; Peckeu, L; Haïk, S

    2013-09-01

    France, involved for a long time in the epidemiological surveillance of transmissible spongiform encephalopathy (TSE), created a national network of surveillance in 1991, because of the description of the first cases of Creutzfeldt-Jakob disease (CJD) linked to a treatment by growth hormone of human origin and the observation of cases of cats infected with the agent of the bovine spongiform encephalopathy in the United Kingdom (UK). The French surveillance network is integrated into the European network of surveillance since its creation in 1993. As in other countries, sporadic CJD is the most frequent form of TSE in France with an annual mortality rate of 1.44 per million. Genetic forms are most often associated with a mutation at codon 200. Among the cases of iatrogenic CJD, 13 cases of CJD after duramater grafts were observed and 119 related to treatment with growth hormone. France is the country worst affected in Europe and the world by this latter form, before the USA and UK. Since 1996, 27 cases of variant of CJD (vCJD) has been observed, making France the second country in the world most affected after the UK. No cases of transfusion-associated vCJD have been observed. Copyright © 2013. Published by Elsevier SAS.

  18. Subtype and regional regulation of prion biomarkers in sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Llorens, Franc; Zafar, Saima; Ansoleaga, Belén; Shafiq, Mohsin; Blanco, Rosi; Carmona, Marga; Grau-Rivera, Oriol; Nos, Carlos; Gelpí, Ellen; Del Río, José Antonio; Zerr, Inga; Ferrer, Isidre

    2015-08-01

    Creutzfeldt-Jakob disease (CJD) is a rapid progressive neurological disease leading to dementia and death. Prion biomarkers are altered in the cerebrospinal fluid (CSF) of CJD patients, but the pathogenic mechanisms underlying these alterations are still unknown. The present study examined prion biomarker levels in the brain and CSF of sporadic CJD (sCJD) cases and their correlation with neuropathological lesion profiles. The expression levels of 14-3-3, Tau, phospho-Tau and α-synuclein were measured in the CSF and brain of sCJD cases in a subtype- and region-specific manner. In addition, the activity of prion biomarker kinases, the expression levels of CJD hallmarks and the most frequent neuropathological sCJD findings were analysed. Prion biomarkers levels were increased in the CSF of sCJD patients; however, correlations between mRNA, total protein and their phosphorylated forms in brain were different. The observed downregulation of the main Tau kinase, GSK3, in sCJD brain samples may help to explain the differential phospho-Tau/Tau ratios between sCJD and other dementias in the CSF. Importantly, CSF biomarkers levels do not necessarily correlate with sCJD neuropathological findings. Present findings indicate that prion biomarkers levels in sCJD tissues and their release into the CSF are differentially regulated following specific modulated responses, and suggest a functional role for these proteins in sCJD pathogenesis. © 2014 British Neuropathological Society.

  19. Creutzfeldt-Jakob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy.

    Science.gov (United States)

    Manix, Marc; Kalakoti, Piyush; Henry, Miriam; Thakur, Jai; Menger, Richard; Guthikonda, Bharat; Nanda, Anil

    2015-11-01

    Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative condition with a rapid disease course and a mortality rate of 100%. Several forms of the disease have been described, and the most common is the sporadic type. The most challenging aspect of this disease is its diagnosis-the gold standard for definitive diagnosis is considered to be histopathological confirmation-but newer tests are providing means for an antemortem diagnosis in ways less invasive than brain biopsy. Imaging studies, electroencephalography, and biomarkers are used in conjunction with the clinical picture to try to make the diagnosis of CJD without brain tissue samples, and all of these are reviewed in this article. The current diagnostic criteria are limited; test sensitivity and specificity varies with the genetics of the disease as well as the clinical stage. Physicians may be unsure of all diagnostic testing available, and may order outdated tests or prematurely request a brain biopsy when the diagnostic workup is incomplete. The authors review CJD, discuss the role of brain biopsy in this patient population, provide a diagnostic pathway for the patient presenting with rapidly progressive dementia, and propose newer diagnostic criteria.

  20. Corticobasal syndrome due to sporadic Creutzfeldt-Jakob disease: a review and neuropsychological case report.

    Science.gov (United States)

    González, David Andrés; Soble, Jason R

    2017-04-01

    Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and fatal neurodegenerative disease with neuropsychological sequelae. This study highlighted a rare presentation of CJD (e.g. corticobasal syndrome [CBS]), reviewed updated diagnostic criteria and procedures for CJD (e.g. diffusion weighted imaging [DWI], real-time quaking-induced conversion [RT-QuIC]), and discussed differential diagnoses. Case report methodology focused on a 68-year-old, Hispanic, right-handed man with 11 years of education. He presented with a 1-2-month history of gait and motor difficulties (e.g. rigidity, myoclonus). After evaluation, a 'cortical ribboning' pattern on DWI and positive RT-QuIC was integrated with performance on neurobehavioral exam (i.e. alien limb phenomenon, unilateral ideomotor apraxia) and neuropsychological testing (i.e. frontal-parietal dysfunction pattern) to reach a diagnosis of sCJD-CBS. The patient expired 3 months after onset of symptoms. This literature review and case report highlighted the importance of staying abreast of developments in neurological literature and the added value of neuropsychology, when integrated with newer procedures, for confirming and excluding diagnostic considerations.

  1. Case series of probable sporadic Creutzfeldt-Jakob disease from Eastern India

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    Atanu Biswas

    2013-01-01

    Full Text Available Background: Creutzfeldt-Jakob disease is a rapidly progressive, fatal, transmissible neurodegenerative disorder caused by prion protein. It is still considered rare in countries like India. This is probably due to nonavailability of autopsy studies in majority of the center. The recent European diagnostic criterion for sporadic CJD (sCJD is useful for making an early diagnosis. Objective: To report a series of patients of probable sCJD from a neurology institute of eastern India. Materials and Methods: Patients of rapidly developing dementia fulfilling the diagnostic criteria for sCJD were included. All were investigated in detail to find out any possible treatable cause including electroencephalography (EEG, magnetic resonance imaging (MRI of brain, and cerebrospinal fluid analysis. Results: A total 10 patients of probable sCJD diagnosed using the European diagnostic criterion between December 2011 and January 2013. The clinical features are consistent with other reported series. While 60% of patients had the classical EEG findings, 100% had typical MRI features. Eight patients died within a mean duration of 4.56 months from the disease onset. Conclusions: The clinical features are similar to other reported series. Our observation raises question about the prevalence of this disease in India which needs more elaborate studies.

  2. Comparative Study of Prions in Iatrogenic and Sporadic Creutzfeldt-Jakob Disease

    Science.gov (United States)

    Xiao, Xiangzhu; Yuan, Jue; Qing, Liuting; Cali, Ignazio; Mikol, Jacqueline; Delisle, Marie-Bernadette; Uro-Coste, Emmanuelle; Zeng, Liang; Abouelsaad, Mai; Gazgalis, Dimitris; Martinez, Manuel Camacho; Wang, Gong-Xian; Brown, Paul; Ironside, James W.; Gambetti, Pierluigi; Kong, Qingzhong; Zou, Wen-Quan

    2014-01-01

    Differentiating iatrogenic Creutzfeldt-Jakob disease (iCJD) from sporadic CJD (sCJD) would be useful for the identification and prevention of human-to-human prion transmission. Currently, the diagnosis of iCJD depends on identification of a recognized source of contamination to which patients have been exposed, in addition to fulfilling basic requirements for the establishment of diagnosis of CJD. Attempts to identify differences in clinical manifestations, neuropathological changes and pathological prion protein (PrPSc) between iCJD and sCJD have been unsuccessful. In the present study, using a variety of more sophisticated methods including sucrose step gradient sedimentation, conformational stability immunoassay, protein misfolding cyclic amplification (PMCA), fragment-mapping, and transmission study, we show no significant differences in gel profiles, oligomeric state, conformational stability and infectivity of PrPSc between iCJD and sCJD. However, using PMCA, we find that convertibility and amplification efficiency of PrPSc is greater in iCJD than in sCJD in a polymorphism-dependent manner. Moreover, two protease-resistant PrP C-terminal fragments (termed PrP-CTF12/13) were detected in all 9 cases of sCJD but not in 6 of 8 cases of iCJD tested in this study. The use of fragment mapping- and PMCA-based assays thus provides a means to distinguish most cases of iCJD from sCJD. PMID:25419482

  3. Short TE quantitative proton magnetic resonance spectroscopy in variant Creutzfeldt-Jakob disease

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    Cordery, R.J.; Godbolt, A.; Rossor, M.N. [University College London, Dementia Research Group, Institute of Neurology, London (United Kingdom); Imperial College of Science, Technology and Medicine, Division of Neuroscience and Psychological Medicine, Faculty of Medicine, London (United Kingdom); MacManus, D. [University College London, NMR Research Unit, Department of Clinical Neurology,Institute of Neurology, London (United Kingdom); Waldman, A.D. [University College London, Dementia Research Group, Institute of Neurology, London (United Kingdom); Imperial College of Science, Technology and Medicine, Division of Neuroscience and Psychological Medicine, Faculty of Medicine, London (United Kingdom); Charing Cross Hospital, Department of Imaging, London (United Kingdom); National Hospital for Neurology and Neurosurgery, Dementia Research Group, London (United Kingdom)

    2006-08-15

    Variant Creutzfeldt-Jakob disease (vCJD) is a fatal neurodegerative disorder. Clinical diagnosis is difficult in the early stages as the disease often presents with non-specific psychiatric and neurological symptoms. To investigate the diagnostic potential of quantitative short TE in vivo MRS, and the nature and anatomical distribution of biochemical abnormalities in vCJD, localised single-voxel spectra (TE/TR 30 ms/2,000 ms) were acquired from three brain regions: thalami, caudate nuclei and frontal white matter. Metabolite concentrations and ratios from three patients with definite or probable vCJD were compared with eight normal age-matched controls. Abnormal signal on T2-weighted MRI was apparent in the pulvinar region in all vCJD patients; this region also showed greatly increased myo-inositol [MI] (mean 2.5-fold, P=0.01) and decreased N-acetyl-aspartate (NAA; mean 2-fold, P=0.01). Two patients also showed increased [MI] (z=17, 11; one with decreased NAA, z=-12) in normal-appearing caudate nuclei. The magnitude of metabolite abnormalities in the thalami in moderately advanced vCJD suggests a potential role in earlier diagnosis. Short TE protocols allow the measurement of MI, which adds discriminant power to the MRS examination. (orig.)

  4. Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999–2008

    Science.gov (United States)

    Hamaguchi, Tsuyoshi; Noguchi-Shinohara, Moeko; Nozaki, Ichiro; Nakamura, Yosikazu; Sato, Takeshi; Kitamoto, Tetsuyuki; Mizusawa, Hidehiro

    2009-01-01

    To elucidate the association between medical procedures and sporadic Creutzfeldt-Jakob disease (sCJD), we analyzed medical procedures (any surgical procedure, neurosurgery, ophthalmic surgery, and blood transfusion) for patients registered by the CJD Surveillance Committee in Japan during 1999–2008. We conducted an age-stratified case–control study with 753 sCJD patients and 210 controls and a study of patients who underwent neurosurgical or ophthalmic surgical procedures at the same hospital. Although the control group was relatively small, no evidence was found that prion disease was transmitted through the investigated medical procedures before onset of sCJD. After onset of sCJD, 4.5% of the sCJD patients underwent operations, including neurosurgical for 0.8% and ophthalmic for 1.9%; no special precautions against transmission of prion diseases were taken. Fortunately, we have not identified patients with prion disease attributed to these operations. Our findings indicate that surgical procedures or blood transfusion had little effect on the incidence of sCJD. PMID:19193271

  5. Cerebrospinal fluid markers in the differentiation of molecular subtypes of sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Gmitterová, K; Heinemann, U; Krasnianski, A; Gawinecka, J; Zerr, I

    2016-06-01

    Cerebrospinal fluid (CSF) analysis supports the clinical diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) when applied within an adequate clinical context. A diagnostic potential has been attributed to CSF proteins such as 14-3-3, but also tau protein, phosphorylated tau (181P) (p-tau) protein, amyloid β1-42 , S100B and neuron-specific enolase (NSE). There has been only limited information available about the contribution of CSF analysis in the differentiation of various molecular sCJD subtypes. The CSF levels of the aforementioned proteins from 73 sCJD patients with distinct molecular subtypes were determined. Differences in tau values were significant amongst the homozygous patients (MM and VV genotype) compared to the heterozygous group (P = 0.07 and P = 0.02 respectively). Significantly higher CSF tau levels (P = 0.003) and NSE (P = 0.02) but lower p-tau/tau ratio (P = 0.01) were observed in MM1 compared to MM2 patients. The p-tau/tau ratio enabled the differentiation of MV genotype with higher levels in PrP(sc) type 2 (P = 0.04). Elevation of S100B (P disease duration and clinical stage influenced the test sensitivity in all proteins. Cerebrospinal fluid protein levels might be useful in the pre-mortem differentiation of molecular sCJD subtypes when the codon 129 genotype is known. © 2016 EAN.

  6. Laminar Distribution of the Pathological Changes in Sporadic and Variant Creutzfeldt-Jakob Disease

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    R. A. Armstrong

    2011-01-01

    Full Text Available The laminar distributions of the pathological changes in the cerebral cortex were compared in the prion diseases sporadic Creutzfeldt-Jakob disease (sCJD and variant CJD (vCJD. First, in some cortical regions, the vacuolation (“spongiform change” was more generally distributed across the cortex in sCJD. Second, there was greater neuronal loss in the upper cortex in vCJD and in the lower cortex in sCJD. Third, the “diffuse” and “florid” prion protein (PrPsc deposits were more frequently distributed in the upper cortex in vCJD and the “synaptic” deposits in the lower cortex in sCJD. Fourth, there was a significant gliosis mainly affecting the lower cortex of both disorders. The data suggest that the pattern of cortical degeneration is different in sCJD and vCJD which may reflect differences in aetiology and the subsequent spread of prion pathology within the brain.

  7. CT and MRI in iatrogenic and sporadic Creutzfeldt-Jakob disease: as far as imaging perseives

    Energy Technology Data Exchange (ETDEWEB)

    Garcia Santos, J.M. [Servicio de Radiodiagnostico, HU Dr. Morales Meseguer, Murcia (Spain)]|[Section of Neuroradiology, HU Virgen de la Arrixaca, Murcia (Spain); Lopez Corbalan, J.A. [Section of Neuroradiology, HU Virgen de la Arrixaca, Murcia (Spain); Martinez-Lage, J.F. [Service of Neurosurgery, HU Virgen de la Arrixaca, Murcia (Spain); Sicilis Guillen, J. [Service of Neurology, HU Virgen de la Arrixaca, Murcia (Spain)

    1996-04-01

    Creutzfeldt-Jakob Disease (CJD), an invariably fatal dementing illness, affects patients in middle and old age (sporadic form). However, the association of CJD with certain treatments (iatrogenic form) has been described in younger patients. The clinical onset of the two forms seems to differ; in the iatrogenic form a high frequency of the ataxic CJD variant has been reported. Nowadays, a definitive diagnosis of CJD is exclusively histological. We present five cases of CJD, one sporadic and the others iatrogenic, following dura mater grafts and analyse their CT and MRI features. CT typically demonstrates brain atrophy, generally progressive, but in sporadic CJD midfield MRI also showed abnormal signal, with predominant deep grey matter involvement. The use of narrow windows with proton-density sequences may reveal subtle cortical signal abnormalities not clearly visible with conventional windows. The early demonstration of these changes, in the appropriate clinical context, may suggest CJD and this supports the use of mid- or high magnetic fields in the diagnosis of CJD and other forms of dementia. In our cases of iatrogenic CJD, low-field MRI did not reveal more than the progressive atrophy displayed by CT, and raises the question on the one hand of possible differences, based on imaging, from the sporadic form, and on the other of the lack of sensitivity of low-field magnets to signal changes in CJD. (orig.)

  8. Factors influencing the survival period in Japanese patients with sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Iwasaki, Yasushi; Akagi, Akio; Mimuro, Maya; Kitamoto, Tetsuyuki; Yoshida, Mari

    2015-10-15

    Although Japanese cases of sporadic Creutzfeldt-Jakob disease (sCJD) generally involve longer survival periods compared to those from other countries, details regarding the factors influencing survival are unclear. To determine the influence of certain factors on survival, we retrospectively assessed 51 Japanese MM1-type sCJD patients with respect to background, clinical course, and disease management. No significant differences were found between men and women, tracheotomy and nontracheotomy patients, or patients treated in public and other types of hospitals. Although the survival period of tube-fed patients was significantly longer than that of patients who were not tube fed, survival of patients fed via a nasal tube did not differ significantly from that of gastrostomy-fed patients. The proportion of tube-fed patients was 68.6% (35/51). Disease duration was not significantly associated with age or year of onset. However, it was associated with time from onset to first recognition of myoclonus, first recognition of periodic sharp-wave complexes on electroencephalogram, and progression to the akinetic mutism state. Mechanical ventilation was not performed for any patient. Because the total disease duration increased in cases with a slowly progressive clinical course as a natural outcome, we concluded that the most crucial factor contributing to the prolonged survival of Japanese sCJD patients was tube feeding once the akinetic mutism state had been reached. Copyright © 2015 Elsevier B.V. All rights reserved.

  9. LGI1 antibody encephalopathy overlapping with sporadic Creutzfeldt-Jakob disease

    Science.gov (United States)

    Kim, Boaz; Yoo, Patrick; Sutherland, Tom; Boyd, Alison; Stehmann, Christiane; McLean, Catriona

    2016-01-01

    Objective: To report a rare case of leucine-rich, glioma inactivated 1 (LGI1) antibody–mediated autoimmune encephalopathy clinically overlapping with pathologically confirmed sporadic Creutzfeldt-Jakob disease (CJD). Methods: The patient was investigated with repeated brain MRI, EEG, CSF examination, whole-body fluorodeoxy-glucose positron emission tomography, genetic analysis of the prion protein gene (PRNP), and extensive serologic screening for paraneoplastic and autoimmune encephalopathy markers. Written informed consent was obtained from the patient's next of kin for access to clinical files for research purposes and for publication. Results: The patient was a 77-year-old man who presented with faciobrachial dystonic seizures (FBDS) secondary to LGI1 antibody–mediated autoimmune encephalopathy, with suggestive MRI findings and a complete response to treatment with combinatorial immunosuppression. Stereotactic biopsy of a nonenhancing T1 hyperintense basal ganglia lesion during the initial FBDS phase, albeit following immunosuppression, did not disclose evidence of lymphocytic inflammation. Following full remission of the FBDS, the patient manifested a rapidly progressive dementia associated with gross motor decline confirmed to be CJD at autopsy (molecular subtype VV3), with no evidence of a pathogenic PRNP mutation. Conclusions: Our patient highlights that these rare diseases are not invariably mutually exclusive and underscores the benefits of comprehensive neuropathologic examination of the brain to achieve an accurate diagnosis, especially in complex cases when the clinical trajectory dramatically deviates and a concomitant disease may need to be conscientiously considered to best explain the new clinical course. PMID:27354985

  10. Comparison Between Sporadic and Misdiagnosed Sporadic Creutzfeldt-Jakob Disease: A Report of Two Cases.

    Science.gov (United States)

    Zhao, Xiongfei; Yu, Yingxin; Zhao, Zhiru; Xu, Jiaping

    2015-06-01

    Definite accurate diagnosis for Creutzfeldt-Jakob disease (CJD) depends on neuropathologic examination of brain biopsy or autopsy. However, transmissible nature makes the invasive examination dangerous. This study was set to determine that the clinical features are for the diagnosis of CJD through a comparison study. We compared clinical features of two cases with initial diagnosis of sporadic CJD. One case was finally diagnosed as definite sporadic CJD. According to World Health Organization diagnosis criteria, the other one, which had been diagnosed as probable sporadic CJD, was confirmed as limbic encephalitis after long-term follow-up. Compared with the case of definite sporadic CJD, the misdiagnosed case did not present typical electroencephalogram (EEG) and diffusion-weighted in magnetic resonance images (DWI) of CJD. However, cerebrospinal fluid in the misdiagnosed patient showed 14-3-3 protein positivity. The patient conditions improved after treatment. Through this case comparison, we conclude that EEG and DWI are necessary for accurate diagnosis of sporadic CJD. Further, long-term follow-up is crucial to diagnosis and treatment of CJD.

  11. CT and MRI in iatrogenic and sporadic Creutzfeldt-Jakob disease: as far as imaging perseives

    International Nuclear Information System (INIS)

    Garcia Santos, J.M.; Lopez Corbalan, J.A.; Martinez-Lage, J.F.; Sicilis Guillen, J.

    1996-01-01

    Creutzfeldt-Jakob Disease (CJD), an invariably fatal dementing illness, affects patients in middle and old age (sporadic form). However, the association of CJD with certain treatments (iatrogenic form) has been described in younger patients. The clinical onset of the two forms seems to differ; in the iatrogenic form a high frequency of the ataxic CJD variant has been reported. Nowadays, a definitive diagnosis of CJD is exclusively histological. We present five cases of CJD, one sporadic and the others iatrogenic, following dura mater grafts and analyse their CT and MRI features. CT typically demonstrates brain atrophy, generally progressive, but in sporadic CJD midfield MRI also showed abnormal signal, with predominant deep grey matter involvement. The use of narrow windows with proton-density sequences may reveal subtle cortical signal abnormalities not clearly visible with conventional windows. The early demonstration of these changes, in the appropriate clinical context, may suggest CJD and this supports the use of mid- or high magnetic fields in the diagnosis of CJD and other forms of dementia. In our cases of iatrogenic CJD, low-field MRI did not reveal more than the progressive atrophy displayed by CT, and raises the question on the one hand of possible differences, based on imaging, from the sporadic form, and on the other of the lack of sensitivity of low-field magnets to signal changes in CJD. (orig.)

  12. Neuropsychological Symptoms in Sporadic Creutzfeldt-Jakob Disease Patients in Germany.

    Science.gov (United States)

    Krasnianski, Anna; Bohling, Geeske T; Heinemann, Uta; Varges, Daniela; Meissner, Bettina; Schulz-Schaeffer, Walter J; Reif, Andreas; Zerr, Inga

    2017-01-01

    The polymorphism at codon 129 of the prion protein gene (PRNP) and the PrPSc types 1 and 2 belong to a molecular classification of sporadic Creutzfeldt-Jakob disease (sCJD) that correlates well with the clinical and neuropathological phenotype of sCJD. The aim of the study was to perform the first detailed evaluation of neuropsychological deficits in a large group of definite sCJD patients with known molecular subtype. We analyzed neuropsychological symptoms in a cohort of 248 sCJD patients with known M129 V polymorphism of PRNP and prion protein type. Neuropsychological symptoms were very frequent in our patients (96%) and occurred as early as in the first third of the disease course. Besides amnesia and impaired attention (89% each), frontal lobe syndrome (75%), aphasia (63%), and apraxia (57%) were the most common neuropsychological deficits. There was no statistically significant difference with regard to frequency of neuropsychological symptoms between the subtypes. In MV2 and VV2 patients, the onset of neuropsychological symptoms was significantly later than in all other subtypes. We provide the first detailed analysis of neuropsychological symptoms in a large group of sCJD patients with known M129 V genotype and prion protein type. We suggest that the rate of progression of neuropsychological symptoms is subtype-specific. These data may improve the diagnosis in atypical sCJD subtypes.

  13. Value of diffusion-weighted MR imaging in the diagnosis of Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Xu Quangang; Wu Weiping; Huang Dehui; Zhang Jiatang; Lang Senyang; Pu Chuanqiang

    2005-01-01

    Objective: To assess the diagnosis value of diffusion- weighted imaging (DWI) in Creutzfeldt-Jakob disease (CJD). Methods: 8 cases of sporadic CJD who underwent MRI were reported. 4 cases were definite, 3 cases were probable and 1 case was possible. The sensitivity of DWI and conventional MRI were compared. Results: T 1 WI and T 2 WI revealed no abnormal signals except nonspecific diffuse brain atrophy in 4 cases, whereas DWI detected hyperintense abnormalities in all cases. 2 cases showed linear lesions only in the cerebral cortex, and 6 cases showed lesions in both the cerebral cortex and the striatum. The lesions were symmetric in 5 cases, but were asymmetric in the other 3 cases. Although fluid- attenuated inversion recovery (FLAIR) imaging also showed cortical hyperintensity in 1 case, the high signal changes were more evident and extensive on DWI. Conclusions: The hyperintense changes in the cerebral cortices and/or striata on DWI are considered characteristic of CJD. DWI is more sensitive than conventional MRI in depicting lesions of CJD and may be an essential tool for the early diagnosis of this disease. (authors)

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  15. Update: Dura Mater Graft-Associated Creutzfeldt-Jakob Disease - Japan, 1975-2017.

    Science.gov (United States)

    Ae, Ryusuke; Hamaguchi, Tsuyoshi; Nakamura, Yosikazu; Yamada, Masahito; Tsukamoto, Tadashi; Mizusawa, Hidehiro; Belay, Ermias D; Schonberger, Lawrence B

    2018-03-09

    Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder that, according to the most well accepted hypothesis (1), is caused by replicating, transmissible, abnormal forms of a host-encoded prion protein (prions). Most CJD cases occur spontaneously (sporadic CJD) or are inherited (genetic CJD). Iatrogenic CJD can occur after exposure to prion-contaminated instruments or products in medical/surgical settings. Cadaveric dura mater graft-associated CJD (dCJD) accounts for a common form of iatrogenic CJD. This report summarizes the epidemiologic features of 154 cases of dCJD identified in Japan during 1975-2017; these cases account for >60% of dCJD cases reported worldwide (1,2). The unusually high prevalence of dCJD in Japan was first reported in 1997 (3). In 2008, a single brand of graft (Lyodura [B. Braun Melsungen AG, Melsungen, Germany]), frequently used as a patch in neurosurgical procedures, was identified as the probable vehicle of transmission (4). No international recall of the implicated Lyodura occurred, the product had a relatively long shelf life, and the grafts were used frequently in Japanese patients with non-life-threatening conditions (4,5). Since 2008, additional cases have been ascertained, reflecting the identification of previously missed cases and the occurrence of new cases with longer latency periods (interval from exposure to symptom onset) for dCJD (up to 30 years), underscoring the importance of maintaining surveillance for dCJD.

  16. Creutzfeldt-Jakob disease lookback study: 21 years of surveillance for transfusion transmission risk.

    Science.gov (United States)

    Crowder, Lauren A; Schonberger, Lawrence B; Dodd, Roger Y; Steele, Whitney R

    2017-08-01

    Transfusion transmission of human prion diseases has been observed for variant Creutzfeldt-Jakob disease (vCJD), but not for the classic forms of prion disease (CJD: sporadic, genetic, and iatrogenic). Although the presence of prions or misfolded prion proteins in blood has been documented in some patients with the most common form of CJD, sporadic CJD, no transfusion-transmitted cases of CJD have been recognized. Since 1995, the American Red Cross has conducted a lookback study of the recipients of blood products from donors who develop CJD to assess the risk of blood-borne CJD transmission in the United States. Blood donors subsequently diagnosed with confirmed or probable CJD were enrolled and the consignees were asked to identify the recipients of their blood products. These donors' transfusion recipients are traced annually with the National Death Index to see if they subsequently die of CJD. To date, 65 CJD donors have been enrolled along with 826 of their blood recipients. These recipients have contributed 3934 person-years of follow-up and no transfusion-transmitted cases of CJD have been recognized. From this study, as well as other epidemiologic studies, there is no evidence of CJD transfusion transmission; this risk remains theoretical. © 2017 AABB.

  17. Creutzfeldt-Jakob Disease Presenting as Expressive Aphasia and Nonconvulsive Status Epilepticus

    Directory of Open Access Journals (Sweden)

    Hafiz B. Mahboob

    2018-01-01

    Full Text Available Creutzfeldt-Jakob disease (CJD, the most common form of human prion diseases, is a fatal condition with a mortality rate reaching 85% within one year of clinical presentation. CJD is characterized by rapidly progressive neurological deterioration in combination with typical electroencephalography (EEG and magnetic resonance imaging (MRI findings and positive cerebrospinal spinal fluid (CSF analysis for 14-3-3 proteins. Unfortunately, CJD can have atypical clinical and radiological presentation in approximately 10% of cases, thus making the diagnosis often challenging. We report a rare clinical presentation of sporadic CJD (sCJD with combination of both expressive aphasia and nonconvulsive status epilepticus. This patient presented with slurred speech, confusion, myoclonus, headaches, and vertigo and succumbed to his disease within ten weeks of initial onset of his symptoms. He had a normal initial diagnostic workup, but subsequent workup initiated due to persistent clinical deterioration revealed CJD with typical MRI, EEG, and CSF findings. Other causes of rapidly progressive dementia and encephalopathy were ruled out. Though a rare condition, we recommend consideration of CJD on patients with expressive aphasia, progressive unexplained neurocognitive decline, and refractory epileptiform activity seen on EEG. Frequent reimaging (MRI, video EEGs and CSF examination might help diagnose this fatal condition earlier.

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

    Directory of Open Access Journals (Sweden)

    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.

  19. Creutzfeldt-Jakob Disease Mimicking Alzheimer Disease and Dementia With Lewy Bodies-Findings of FDG PET With 3-Dimensional Stereotactic Surface Projection.

    Science.gov (United States)

    Miyazawa, Nobuhiko

    2017-05-01

    A 78-year-old man received a diagnosis of sporadic Creutzfeldt-Jakob disease based on symptoms and findings of MRI, FDG PET, and cerebrospinal fluid markers. PET with 3-dimensional stereotactic surface projection (3D-SSP) showed that the distribution of hypometabolism mimicked that of Alzheimer disease. A 68-year-old woman was treated under a diagnosis of convulsion. Findings of MRI, PET, familial history, and cerebrospinal fluid markers revealed familial Creutzfeldt-Jakob disease. FDG PET with 3D-SSP disclosed that the hypometabolic pattern mimicked that of dementia with Lewy bodies. FDG PET with 3D-SSP can demonstrate similar patterns in various neurodegenerative disorders.

  20. Positive 14-3-3 and tau proteins in a sporadic Creutzfeldt-Jakob disease case and a brief perspective of prion diseases in Colombia.

    Science.gov (United States)

    Escandón-Vargas, Kevin; Zorrilla-Vaca, Andrés; Corral-Prado, Raúl Heli

    2016-02-24

    Prion diseases are rare neurodegenerative disorders occurring worldwide and affecting both humans and animals. Herein, we present the case of a patient diagnosed with definite sporadic Creutzfeldt-Jakob disease in Cali, Colombia. Besides neurological examination, 14-3-3 and tau proteins were valuable tools supporting the diagnosis. We also present a brief perspective of the prion diseases reported in Colombia to date. Although the incidence of prion diseases is unknown in Colombia, our literature review revealed that one case of scrapie in 1981 and 29 human sporadic cases of Creutzfeldt-Jakob disease have been documented and published in our country.

  1. The value of magnetic resonance imaging in the early diagnosis of Creutzfeldt-Jakob disease – own experience

    International Nuclear Information System (INIS)

    Bekiesińska-Figatowska, Monika; Kuczyńska-Zardzewiały, Arleta; Pomianowska, Barbara; Kajdana, Katarzyna; Szpak, Grażyna M.; Iwanowska, Beata; Mądzik, Jarosław

    2012-01-01

    Creutzfeldt-Jakob disease (CJD) is a rare progressive neurodegenerative disorder, caused by the deposition of the pathological isoform of prion protein PrPsc in the central nervous system. The classic triad of symptoms consists of: rapidly progressive dementia, myoclonus and typical electroencephalographic findings (intermittent rhythmic delta activity and periodic sharp wave complexes). Detection of 14-3-3 protein in the cerebrospinal fluid plays an important diagnostic role as well. Magnetic resonance (MR) images of the brain have been recently incorporated into the diagnostic criteria of sporadic Creutzfeldt-Jakob disease. MR examinations were performed in a 65-year-old man and a 54-year-old woman with delusional disorder and cognitive dysfunction, respectively. Diffusion restriction (hyperintense signal in DWI) was shown in the cortex of the left parietal and occipital lobe in the first patient and symmetrically in the cortex of both cerebral hemispheres except for precentral gyri in the second one. In both cases, the first examinations were misread, with the suspicion of ischemic infarcts as the first differential diagnosis. Consultations and subsequent MR examinations in which lesions in subcortical nuclei appeared allowed for a diagnosis of probable CJD. In the first case it was confirmed by clinical picture, EEG and finally – autopsy. In the second case, EEG was not typical for CJD but the clinical course of the disease confirmed that diagnosis. The authors present the cases of two patients with characteristic MR images that allowed early diagnosis of probable Creutzfeldt-Jakob disease before the characteristic clinical picture appeared. Early diagnosis is nowadays important for the prevention of disease transmission and in the future – hopefully – for early treatment

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

    Directory of Open Access Journals (Sweden)

    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.

  3. Validation of α-Synuclein as a CSF Biomarker for Sporadic Creutzfeldt-Jakob Disease.

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    Llorens, Franc; Kruse, Niels; Karch, André; Schmitz, Matthias; Zafar, Saima; Gotzmann, Nadine; Sun, Ting; Köchy, Silja; Knipper, Tobias; Cramm, Maria; Golanska, Ewa; Sikorska, Beata; Liberski, Pawel P; Sánchez-Valle, Raquel; Fischer, Andre; Mollenhauer, Brit; Zerr, Inga

    2018-03-01

    The analysis of cerebrospinal fluid (CSF) biomarkers gains importance in the differential diagnosis of prion diseases. However, no single diagnostic tool or combination of them can unequivocally confirm prion disease diagnosis. Electrochemiluminescence (ECL)-based immunoassays have demonstrated to achieve high diagnostic accuracy in a variety of sample types due to their high sensitivity and dynamic range. Quantification of CSF α-synuclein (a-syn) by an in-house ECL-based ELISA assay has been recently reported as an excellent approach for the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD), the most prevalent form of human prion disease. In the present study, we validated a commercially available ECL-based a-syn ELISA platform as a diagnostic test for correct classification of sCJD cases. CSF a-syn was analysed in 203 sCJD cases with definite diagnosis and in 445 non-CJD cases. We investigated reproducibility and stability of CSF a-syn and made recommendations for its analysis in the sCJD diagnostic workup. A sensitivity of 98% and a specificity of 97% were achieved when using an optimal cut-off of 820 pg/mL a-syn. Moreover, we were able to show a negative correlation between a-syn levels and disease duration suggesting that CSF a-syn may be a good prognostic marker for sCJD patients. The present study validates the use of a-syn as a CSF biomarker of sCJD and establishes the clinical and pre-analytical parameters for its use in differential diagnosis in clinical routine. Additionally, the current test presents some advantages compared to other diagnostic approaches: it is fast, economic, requires minimal amount of CSF and a-syn levels are stable along disease progression.

  4. Possible iatrogenic transmission of Creutzfeldt-Jakob disease via tonometer tips: a review of the literature.

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    Walia, J S; Chronister, C L

    2001-10-01

    Tonometer tips are used by optometrists to measure intraocular pressures. The recommended procedure of soaking in bleach solution kills bacteria and certain viruses, such as human immunodeficiency virus, herpes simplex virus-1 and herpes simplex virus-2, adenovirus 8, and hepatitis B, from the tip. Conversely, recommendations made in literature to sterilize equipment that may have come in contact with virus-contaminated tissue from patients with Creutzfeldt-Jakob disease have a somewhat tougher requirement. Autoclaving for 1 hour at a temperature of at least 120 degrees C (15 psi), or a 1-hour exposure to 0.5% sodium hypochlorite (a 10-fold dilution of household bleach) should provide excellent disinfection. One-hour exposure to 1 N Sodium hydroxide has also been mentioned in the literature. Studies have shown that corneas of guinea pigs with Cruetzfeldt-Jakob disease (C-J disease) are infectious. Infected corneas have been shown to cause transmission via corneal transplants, and via experimental placement of infected guinea pig's cornea into the anterior chamber of uninfected guinea pigs. Many researchers have strongly suggested that C-J disease can be iatrogenically transmitted via applanation tonometer tips. An epidemiologic case-controlled study found statistically significant odds ratio for intraocular pressure testing in the medical history of patients with C-J disease. Even though there have not been any proven studies confirming iatrogenic transmission through tonometer tips, optometrists should be cautious if a patient has C-J disease, or manifests symptoms of C-J disease and use alternatives to Goldmann applanation tonometry.

  5. Transmission of sporadic Creutzfeldt-Jakob disease by blood transfusion: risk factor or possible biases.

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    Puopolo, Maria; Ladogana, Anna; Vetrugno, Vito; Pocchiari, Maurizio

    2011-07-01

    The occurrence of transfusion transmissions of variant Creutzfeldt-Jakob disease (CJD) cases has reawakened attention to the possible similar risk posed by other forms of CJD. CJD with a definite or probable diagnosis (sporadic CJD, n = 741; genetic CJD, n = 175) and no-CJD patients with definite alternative diagnosis (n = 482) with available blood transfusion history were included in the study. The risk of exposure to blood transfusion occurring more than 10 years before disease onset and for some possible confounding factors was evaluated by calculating crude odds ratios (ORs). Variables with significant ORs in univariate analyses were included in multivariate logistic regression analyses. In the univariate model, blood transfusion occurring more than 10 years before clinical onset is 4.1-fold more frequent in sporadic CJD than in other neurologic disorders. This significance is lost when the 10-year lag time was not considered. Multivariate analyses show that the risk of developing sporadic CJD after transfusion increases (OR, 5.05) after adjusting for possible confounding factors. Analysis conducted on patients with genetic CJD did not reveal any significant risk factor associated with transfusion. This is the first case-control study showing a significant risk of transfusion occurring more than 10 years before clinical onset in sporadic CJD patients. It remains questionable whether the significance of these data is biologically plausible or the consequence of biases in the design of the study, but they counterbalance previous epidemiologic negative reports that might have overestimated the assessment of blood safety in sporadic CJD. © 2010 American Association of Blood Banks.

  6. Progressive Stroke-Like Symptoms in a Patient with Sporadic Creutzfeldt-Jakob Disease

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    Jukka Lyytinen

    2010-03-01

    Full Text Available Sporadic Creutzfeldt-Jakob disease (sCJD is a rare neurodegenerative disorder in which accumulation of a pathogenic isoform of prion protein (PrPSc induces neuronal damage with distinct pathologic features. The prognosis of sCJD is devastating: rapid clinical decline is followed by death generally within months after onset of symptoms. The classic clinical manifestations of sCJD are rapidly progressing dementia, myoclonus, and ataxia. However, the spectrum of clinical features can vary considerably. We describe a definite, neuropathologically verified sCJD in a 67-year-old woman who initially presented with progressive stroke-like symptoms: left-sided hemiparesis and ataxia within a few days. The initial brain magnetic resonance imaging (MRI showed bilateral cortical hyperintensity on diffusion-weighted sequences (DWI resembling multiple ischemic lesions. Despite anticoagulation with low-molecular-weight heparin, the patient deteriorated rapidly, became dysphagic and bedridden with myoclonic jerks on her left side extremities correlating with intermittent high-amplitude epileptiform discharges on electroencephalography (EEG. Basal ganglia hyperintense signal changes in addition to cortical ribboning were seen in DWI images of a follow-up MRI. Repeated EEG recordings showed an evolution to periodic sharp wave complexes. Protein 14-3-3 was positive in her cerebrospinal fluid specimen, in addition to an abnormally high total tau level. In the terminal stage the patient was in an akinetic, mutistic state with deteriorating consciousness. She died 19 days after admission to the hospital. Neuropathologic investigation corroborated the clinical diagnosis of sCJD with spongiform degeneration and immunohistochemical demonstration of the deposition of pathologic PrPSc.

  7. Fatal Prion Disease in a Mouse Model of Genetic E200K Creutzfeldt-Jakob Disease

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    Friedman-Levi, Yael; Meiner, Zeev; Canello, Tamar; Frid, Kati; Kovacs, Gabor G.; Budka, Herbert; Avrahami, Dana; Gabizon, Ruth

    2011-01-01

    Genetic prion diseases are late onset fatal neurodegenerative disorders linked to pathogenic mutations in the prion protein-encoding gene, PRNP. The most prevalent of these is the substitution of Glutamate for Lysine at codon 200 (E200K), causing genetic Creutzfeldt-Jakob disease (gCJD) in several clusters, including Jews of Libyan origin. Investigating the pathogenesis of genetic CJD, as well as developing prophylactic treatments for young asymptomatic carriers of this and other PrP mutations, may well depend upon the availability of appropriate animal models in which long term treatments can be evaluated for efficacy and toxicity. Here we present the first effective mouse model for E200KCJD, which expresses chimeric mouse/human (TgMHu2M) E199KPrP on both a null and a wt PrP background, as is the case for heterozygous patients and carriers. Mice from both lines suffered from distinct neurological symptoms as early as 5–6 month of age and deteriorated to death several months thereafter. Histopathological examination of the brain and spinal cord revealed early gliosis and age-related intraneuronal deposition of disease-associated PrP similarly to human E200K gCJD. Concomitantly we detected aggregated, proteinase K resistant, truncated and oxidized PrP forms on immunoblots. Inoculation of brain extracts from TgMHu2ME199K mice readily induced, the first time for any mutant prion transgenic model, a distinct fatal prion disease in wt mice. We believe that these mice may serve as an ideal platform for the investigation of the pathogenesis of genetic prion disease and thus for the monitoring of anti-prion treatments. PMID:22072968

  8. Fatal prion disease in a mouse model of genetic E200K Creutzfeldt-Jakob disease.

    Directory of Open Access Journals (Sweden)

    Yael Friedman-Levi

    2011-11-01

    Full Text Available Genetic prion diseases are late onset fatal neurodegenerative disorders linked to pathogenic mutations in the prion protein-encoding gene, PRNP. The most prevalent of these is the substitution of Glutamate for Lysine at codon 200 (E200K, causing genetic Creutzfeldt-Jakob disease (gCJD in several clusters, including Jews of Libyan origin. Investigating the pathogenesis of genetic CJD, as well as developing prophylactic treatments for young asymptomatic carriers of this and other PrP mutations, may well depend upon the availability of appropriate animal models in which long term treatments can be evaluated for efficacy and toxicity. Here we present the first effective mouse model for E200KCJD, which expresses chimeric mouse/human (TgMHu2M E199KPrP on both a null and a wt PrP background, as is the case for heterozygous patients and carriers. Mice from both lines suffered from distinct neurological symptoms as early as 5-6 month of age and deteriorated to death several months thereafter. Histopathological examination of the brain and spinal cord revealed early gliosis and age-related intraneuronal deposition of disease-associated PrP similarly to human E200K gCJD. Concomitantly we detected aggregated, proteinase K resistant, truncated and oxidized PrP forms on immunoblots. Inoculation of brain extracts from TgMHu2ME199K mice readily induced, the first time for any mutant prion transgenic model, a distinct fatal prion disease in wt mice. We believe that these mice may serve as an ideal platform for the investigation of the pathogenesis of genetic prion disease and thus for the monitoring of anti-prion treatments.

  9. Early pathology in sleep studies of patients with familial Creutzfeldt-Jakob disease.

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    Givaty, Gili; Maggio, Nicola; Cohen, Oren S; Blatt, Ilan; Chapman, Joab

    2016-10-01

    In this study, we aimed to assess sleep function in patients with recent-onset familial Creutzfeldt-Jakob disease (fCJD). The largest cluster of fCJD patients is found in Jews of Libyan origin, linked to the prion protein gene (PRNP) E200K mutation. The high index of suspicion in these patients often leads to early diagnosis, with complaints of insomnia being a very common presenting symptom of the disease. The study included 10 fCJD patients diagnosed by clinical manifestations, magnetic resonance imaging (MRI) scan of the brain, elevated tau protein in the cerebrospinal fluid (CSF) and positive PRNP E200K mutation. Standard polysomnography was performed after a brief interview confirming the presence of sleep disturbances. All patients showed a pathological sleep pattern according to all scoring evaluation settings. The sleep stages were characterized by (i) disappearance of sleep spindles; (ii) outbursts of periodic sharp waves and shallowing of sleep consisting in increased Stage 2 and wake periods during the night, as well as decrease of slow-wave sleep and rapid eye movement (REM) sleep. Recordings of respiratory functions reported irregular breathing with central and obstructive apnea and hypopnea. The typical hypotonia occurring during the night and atonia during REM sleep were replaced by hyperactive sleep consisting of multiple jerks, movements and parasomnia (mainly talking) throughout the night. In conclusion, we report unique pathological sleep patterns in early fCJD associated with the E200K mutation. Specific respiratory disturbances and lack of atonia could possibly serve as new, early diagnostic tools in the disease. © 2016 European Sleep Research Society.

  10. Clinical radiological correlation in E200K familial Creutzfeldt-Jakob disease.

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    Cohen, Oren S; Chapman, Joab; Korczyn, Amos D; Siaw, Oliver L; Warman-Alaluf, Naama; Nitsan, Zeev; Appel, Shmuel; Kahana, Esther; Rosenmann, Hanna; Hoffmann, Chen

    2016-12-01

    The use of diffusion MRI improved the accuracy of diagnosis in Creutzfeldt-Jakob disease (CJD) and expanded our knowledge of the changes occurring in the brain during the disease. The aim of this study was to test whether in patients with E200K familial CJD (fCJD) the clinical severity correlates with the disease burden as reflected by the extent of cortical involvement in DWI MRI. Consecutive fCJD patients were examined by a neurologist who performed several tests including the CJD neurological scale (CJD-NS), MiniMental status examination (MMSE), Frontal Assessment Battery (FAB), NIH Stroke Scale (NIHSS), and the expanded disability status scale (EDSS). A simultaneously acquired MRI was analyzed by measuring the extent of cortical involvement in the DWI axial sequence. Correlations were tested for using Pearson test. Fifty-two fCJD patients (35 males, mean age 59.4 ± 5.7 years) were recruited to the study. Significant negative correlation was found between the extent of cortical involvement and the cognitive performance of the patients as reflected by their MMSE and FAB scores. In addition, a significant positive correlation was found between the MRI and the clinical disease severity scales CJD-NS and EDSS. The correlation between clinical scales of severity and cognitive dysfunction and the disease burden confirms the reliability of the CJD-NS scale. Further studies are warranted to examine whether MRI may serve not only for diagnosis but also as a biomarker for follow-up of disease progression and the efficacy of potential treatments.

  11. Unusual presentations in patients with E200K familial Creutzfeldt-Jakob disease.

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    Cohen, O S; Kimiagar, I; Korczyn, A D; Nitsan, Z; Appel, S; Hoffmann, C; Rosenmann, H; Kahana, E; Chapman, J

    2016-05-01

    Familial Creutzfeldt-Jakob disease (fCJD) in Jews of Libyan ancestry is caused by an E200K mutation in the PRNP gene. The typical presenting symptoms include cognitive decline, behavioral changes and gait disturbances; however, some patients may have an unusual presentation such as a stroke-like presentation, alien hand syndrome or visual disturbances. The aim of this paper is to describe uncommon presentations in our series of consecutive patients with E200K fCJD. The study group included consecutive fCJD patients followed up as part of a longitudinal prospective study ongoing since 2003 or hospitalized since 2005. The clinical diagnosis of probable CJD was based on accepted diagnostic criteria and supported by typical magnetic resonance imaging, electroencephalographic findings, elevated cerebrospinal fluid tau protein levels and by genetic testing for the E200K mutation. Disease symptoms and signs were retrieved from the medical files. The study population included 77 patients (42 men) with a mean age of disease onset of 60.6 ± 7.2 years. The most prevalent presenting symptoms were cognitive decline followed by gait impairment and behavioral changes. However, six patients had an unusual presentation including auditory agnosia, monoparesis, stroke-like presentation, facial nerve palsy, pseudobulbar syndrome and alien hand syndrome. Our case series illustrates the wide phenotypic variability of the clinical presentation of patients with fCJD and widens the clinical spectrum of the disease. A high level of clinical suspicion may prove useful in obtaining early diagnosis and therefore avoiding costly and inefficient diagnostic and therapeutic strategies. © 2016 EAN.

  12. The Distribution of Prion Protein Allotypes Differs Between Sporadic and Iatrogenic Creutzfeldt-Jakob Disease Patients.

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    Moore, Roger A; Head, Mark W; Ironside, James W; Ritchie, Diane L; Zanusso, Gianluigi; Choi, Young Pyo; Pyo Choi, Young; Priola, Suzette A

    2016-02-01

    Sporadic Creutzfeldt-Jakob disease (sCJD) is the most prevalent of the human prion diseases, which are fatal and transmissible neurodegenerative diseases caused by the infectious prion protein (PrP(Sc)). The origin of sCJD is unknown, although the initiating event is thought to be the stochastic misfolding of endogenous prion protein (PrP(C)) into infectious PrP(Sc). By contrast, human growth hormone-associated cases of iatrogenic CJD (iCJD) in the United Kingdom (UK) are associated with exposure to an exogenous source of PrP(Sc). In both forms of CJD, heterozygosity at residue 129 for methionine (M) or valine (V) in the prion protein gene may affect disease phenotype, onset and progression. However, the relative contribution of each PrP(C) allotype to PrP(Sc) in heterozygous cases of CJD is unknown. Using mass spectrometry, we determined that the relative abundance of PrP(Sc) with M or V at residue 129 in brain specimens from MV cases of sCJD was highly variable. This result is consistent with PrP(C) containing an M or V at residue 129 having a similar propensity to misfold into PrP(Sc) thus causing sCJD. By contrast, PrP(Sc) with V at residue 129 predominated in the majority of the UK human growth hormone associated iCJD cases, consistent with exposure to infectious PrP(Sc) containing V at residue 129. In both types of CJD, the PrP(Sc) allotype ratio had no correlation with CJD type, age at clinical onset, or disease duration. Therefore, factors other than PrP(Sc) allotype abundance must influence the clinical progression and phenotype of heterozygous cases of CJD.

  13. 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.

  14. Neuropathological and biochemical criteria to identify acquired Creutzfeldt-Jakob disease among presumed sporadic cases.

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    Kobayashi, Atsushi; Parchi, Piero; Yamada, Masahito; Mohri, Shirou; Kitamoto, Tetsuyuki

    2016-06-01

    As an experimental model of acquired Creutzfeldt-Jakob disease (CJD), we performed transmission studies of sporadic CJD using knock-in mice expressing human prion protein (PrP). In this model, the inoculation of the sporadic CJD strain V2 into animals homozygous for methionine at polymorphic codon 129 (129 M/M) of the PRNP gene produced quite distinctive neuropathological and biochemical features, that is, widespread kuru plaques and intermediate type abnormal PrP (PrP(Sc) ). Interestingly, this distinctive combination of molecular and pathological features has been, to date, observed in acquired CJD but not in sporadic CJD. Assuming that these distinctive phenotypic traits are specific for acquired CJD, we revisited the literature and found two cases showing widespread kuru plaques despite the 129 M/M genotype, in a neurosurgeon and in a patient with a medical history of neurosurgery without dura mater grafting. By Western blot analysis of brain homogenates, we revealed the intermediate type of PrP(Sc) in both cases. Furthermore, transmission properties of brain extracts from these two cases were indistinguishable from those of a subgroup of dura mater graft-associated iatrogenic CJD caused by infection with the sporadic CJD strain V2. These data strongly suggest that the two atypical CJD cases, previously thought to represent sporadic CJD, very likely acquired the disease through exposure to prion-contaminated brain tissues. Thus, we propose that the distinctive combination of 129 M/M genotype, kuru plaques, and intermediate type PrP(Sc) , represents a reliable criterion for the identification of acquired CJD cases among presumed sporadic cases. © 2015 Japanese Society of Neuropathology.

  15. Role of the biomarkers for the diagnosis of Creutzfeldt-Jakob disease.

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    Dulamea, A; Solomon, E

    2016-01-01

    Sporadic Creutzfeldt-Jakob disease (CJD) is a human prion disease, rapidly progressive and fatal, characterized by spongiform encephalopathy. The characteristic triad of signs - rapidly progressive dementia, myoclonus and periodic sharp wave complexes (PSWC) on electroencephalography (EEG) - usually appear in the late stages of the disease. The clinical diagnosis of CJD ante-mortem involves the exclusion of the rapidly progressive non-prionic dementias, the definitive diagnosis requiring brain tissue confirmation. Authors evaluated the methods of clinical diagnosis for sporadic CJD. This study retrospectively reviewed the medical records of patients diagnosed with probable sporadic CJD, based on brain magnetic resonance imaging (MRI), EEG, cerebrospinal fluid (CSF) analysis and extensive laboratory work-up. Four patients with a mean age of 67 years were included in our study. The mean duration from diagnosis until death was of 3.2 weeks. The clinical features of the disease at onset were atypical. In the final stage of the disease, all patients presented rapidly progressive dementia and myoclonus. High levels of 14-3-3 protein and tau protein and normal levels of amyloid β1-42 were found at CSF analysis, in all patients. PSWC on EEG were present in 3 out of 4 patients at different moments of the disease. MRI showed hyperintense lesions in brain cortex, caudate nucleus, and putamen on T2, FLAIR, and DWI. CJD may present various clinical features and, since brain biopsy is usually difficult to perform, a combination of biomarkers is useful in order to establish the diagnosis in the early phase of the disease.

  16. Redefining periodic patterns on electroencephalograms of patients with sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Shin, Jung-Won; Yim, Byeongsoo; Oh, Seung Hun; Kim, Nam Keun; Lee, Sang Kun; Kim, Ok-Joon

    2017-05-01

    We aimed to redefine various periodic patterns (PPs) observed on electroencephalography (EEG) in patients with sporadic Creutzfeldt-Jakob disease (sCJD) using the American Clinical Neurophysiology Society's (ACNS) Criteria. We analyzed EEG data of 23 patients with sCJD were admitted to two university hospitals between August 2005 and September 2015. We classified PPs on EEG data into three types: irregular periodic discharges (PDs) with superimposed rhythmic activities, appearing at a median of 8weeks after onset (w.a.o.); rhythmic sharp-and-wave, at a median of 11w.a.o.; and PDs with biphasic or triphasic morphology, at a median of 17w.a.o. Of 16 patients presenting with PPs, 14 had widespread lesions in both cortical and subcortical areas with clinical stage III at admission, and shorter time intervals for admission to hospital from disease onset than patients without PPs (Patients with PP, 11.6±12.2weeks; without PP, 18.2±8.3weeks; p=0.033). PPs largely presented as three types at different stages of disease progression, and patients who had PPs had more wide spread lesions and rapid disease progression. Our redefinition of PPs demonstrated on EEG using the ACNS criteria may contribute to further understanding of the pathological mechanisms of sCJD, and PPs might be a predictive factor of a rapid sCJD progression. Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

  17. Seizures in E200K familial and sporadic Creutzfeldt-Jakob disease.

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    Appel, S; Chapman, J; Cohen, O S; Rosenmann, H; Nitsan, Z; Blatt, I

    2015-03-01

    Although seizures (other than myoclonus) are frequently reported in Creutzfeldt-Jakob disease (CJD), their frequency, clinical manifestations, and effect on the disease course is unknown. To characterize the frequency of seizures in E200K familial and sporadic CJD, to describe its semiology, EEG and MRI findings. In this retrospective study, we reviewed all patients with CJD who were seen in the Sheba Medical Center between the years 2003-2012 and underwent clinical evaluation, genetic testing, EEG and MRI studies. The diagnosis of seizures was carried out based on documentation of episodes consistent with seizures or episode of unresponsiveness correlated with ictal activity in EEG. Sixty-four probable patients with CJD were included in the study, 57 (89%) with E200K familial (fCJD) and 7 (11%) with sporadic (sCJD). Seizures occurred in 8 patients: 3 of 7 (43%) in patients with sCJD compared to 5/57 (9%) in patients with E200K fCJD (P = 0.04, chi-square test). Two of E200K fCJD patients with seizures had other non-prion etiologies for seizures (brain metastasis, known history of temporal lobe epilepsy which started 44 years before the diagnosis of CJD). Seizures occurred late in the course of the disease with an average of 12 days between the onset of seizures and death. Seizures in E200K fCJD were infrequent and occurred late in the disease course. This difference suggests that E200K fCJD represents a separate subtype of the disease with distinct clinical characteristics. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

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

    Science.gov (United States)

    Mader, Edward C.; El-Abassi, Rima; Villemarette-Pittman, Nicole R.; Santana-Gould, Lenay; Olejniczak, Piotr W.; England, John D.

    2013-01-01

    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. Electroence-phalography (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. PMID:23717780

  19. Specific clinical signs and symptoms are predictive of clinical course in sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Nakatani, E; Kanatani, Y; Kaneda, H; Nagai, Y; Teramukai, S; Nishimura, T; Zhou, B; Kojima, S; Kono, H; Fukushima, M; Kitamoto, T; Mizusawa, H

    2016-09-01

    Akinetic mutism is thought to be an appropriate therapeutic end-point in patients with sporadic Creutzfeldt-Jakob disease (sCJD). However, prognostic factors for akinetic mutism are unclear and clinical signs or symptoms that precede this condition have not been defined. The goal of this study was to identify prognostic factors for akinetic mutism and to clarify the order of clinical sign and symptom development prior to its onset. The cumulative incidence of akinetic mutism and other clinical signs and symptoms was estimated based on Japanese CJD surveillance data (455 cases) collected from 2003 to 2008. A proportional hazards model was used to identify prognostic factors for the time to onset of akinetic mutism and other clinical signs and symptoms. Periodic synchronous discharges on electroencephalography were present in the majority of cases (93.5%). The presence of psychiatric symptoms or cerebellar disturbance at sCJD diagnosis was associated with the development of akinetic mutism [hazard ratio (HR) 1.50, 95% confidence interval (CI) 1.14-1.99, and HR 2.15, 95% CI1.61-2.87, respectively]. The clinical course from cerebellar disturbance to myoclonus or akinetic mutism was classified into three types: (i) direct path, (ii) path via pyramidal or extrapyramidal dysfunction and (iii) path via psychiatric symptoms or visual disturbance. The presence of psychiatric symptoms or cerebellar disturbance increased the risk of akinetic mutism of sCJD cases with probable MM/MV subtypes. Also, there appear to be sequential associations in the development of certain clinical signs and symptoms of this disease. © 2016 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.

  20. Beyond PrPres Type 1/Type 2 Dichotomy in Creutzfeldt-Jakob Disease

    Science.gov (United States)

    Simon, Stéphanie; Lugan, Séverine; Bilheude, Jean-Marc; Perret-Liaudet, Armand; Ironside, James W.; Haik, Stéphane; Basset-Leobon, Christelle; Lacroux, Caroline; Peoch', Katell; Streichenberger, Nathalie; Langeveld, Jan; Head, Mark W.; Grassi, Jacques; Hauw, Jean-Jacques; Schelcher, Francois; Delisle, Marie Bernadette; Andréoletti, Olivier

    2008-01-01

    Sporadic Creutzfeldt-Jakob disease (sCJD) cases are currently subclassified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the proteinase K (PK) digested abnormal prion protein (PrPres) identified on Western blotting (type 1 or type 2). These biochemically distinct PrPres types have been considered to represent potential distinct prion strains. However, since cases of CJD show co-occurrence of type 1 and type 2 PrPres in the brain, the basis of this classification system and its relationship to agent strain are under discussion. Different brain areas from 41 sCJD and 12 iatrogenic CJD (iCJD) cases were investigated, using Western blotting for PrPres and two other biochemical assays reflecting the behaviour of the disease-associated form of the prion protein (PrPSc) under variable PK digestion conditions. In 30% of cases, both type 1 and type 2 PrPres were identified. Despite this, the other two biochemical assays found that PrPSc from an individual patient demonstrated uniform biochemical properties. Moreover, in sCJD, four distinct biochemical PrPSc subgroups were identified that correlated with the current sCJD clinico-pathological classification. In iCJD, four similar biochemical clusters were observed, but these did not correlate to any particular PRNP 129 polymorphism or western blot PrPres pattern. The identification of four different PrPSc biochemical subgroups in sCJD and iCJD, irrespective of the PRNP polymorphism at codon 129 and the PrPres isoform provides an alternative biochemical definition of PrPSc diversity and new insight in the perception of Human TSE agents variability. PMID:18389084

  1. The sensitivity of auxiliary examinations in different stages of sporadic Creutzfeldt-Jakob disease

    Directory of Open Access Journals (Sweden)

    Jiao-jiao JIANG

    2017-06-01

    Full Text Available Objective To analyze the sensitivity of auxiliary examinations in different periods of sporadic Creutzfeldt-Jakob disease (sCJD. Methods The clinical data of 53 sCJD patients were retrospectively analyzed including the different stages of skull diffusion-weighted magnetic resonance imaging (DWI, 24-hour ambulatory electroencephalogram (EEG, 18F-FDG PET/CT (PET-CT and cerebrospinal fluid 14-3-3 protein. When calculating the sensitivity of an auxiliary examination, the diagnostic criteria were defined by combining the specific clinical manifestations with two or more positive results of other auxiliary examinations. Results There were 24, 53 and 22 sCJD patients, respectively, met the criterion of early (E, middle (M and later (L stage of disease (some patients fit 2 or 3 stages. The sensitivity of DWI (E: 58.3%, M: 85.4%, L: 94.7%, EEG (E: 45.8%, M: 62.7%, L: 77.8%, 14-3-3 protein in cerebrospinal fluid (E: 11.1%, M: 52.9% and PET-CT (E: 80%, M: 100% increased gradually with disease progression. The sensitivity of PET-CT was higher than the other auxiliary examinations for E and M stages; no PET-CT was conducted in L stage. High signal regions mainly distributed in the cortex in E and M stages, but in L stage, no significant difference was found on the distribution of high signal regions between cortex and basal ganglia. Conclusions The sensitivities of the auxiliary examinations were different for sCJD patients in different stages. Reexaminations in different periods may improve the sensitivity for sCJD diagnosis. The sensitivity of PET-CT was high, and the combination of PET-CT and other auxiliary examinations may play a key role in the diagnosis of sCJD. DOI: 10.11855/j.issn.0577-7402.2017.05.15

  2. Caregiver burden in atypical dementias: comparing frontotemporal dementia, Creutzfeldt-Jakob disease, and Alzheimer's disease.

    Science.gov (United States)

    Uflacker, Alice; Edmondson, Mary C; Onyike, Chiadi U; Appleby, Brian S

    2016-02-01

    Caregiver burden is a significant issue in the treatment of dementia and a known contributor to institutionalization of patients with dementia. Published data have documented increased caregiver burden in behavioral variant frontotemporal dementia (bvFTD) compared to Alzheimer's disease (AD). Another atypical dementia with high-perceived caregiver burden is sporadic Creutzfeldt-Jakob disease (sCJD), but no formal studies have assessed this perception. The aim of this study was to compare caregiver burden across atypical dementia etiologies. 76 adults with atypical dementia (young-onset AD [YOAD], bvFTD, language variant FTD [lvFTD], and sCJD) were administered an abbreviated version of the Zarit Burden Interview (ZBI), Neuropsychiatric Inventory (NPI-Q), and other assessment instruments during a five-year time period at Johns Hopkins Hospital (JHH). A Cox regression model examined differences between disease categories that impact mean ZBI scores. Mean ZBI scores were significantly different between dementia etiologies, with bvFTD and sCJD having the highest caregiver burden (p = 0.026). Mean NPI-Q caregiver distress scores were highest in bvFTD and sCJD (p = 0.002), with sCJD and bvFTD also having the highest number of endorsed symptom domains (p = 0.012). On regression analyses, an interactive variable combining final diagnosis category and NPI-Q total severity score demonstrated statistically significant differences in mean ZBI scores for sCJD and bvFTD. This study demonstrates that bvFTD and sCJD have increased levels of caregiver burden, NPI-Q caregiver distress, total severity scores, and number of endorsed symptom domains. These results suggest that higher caregiver burden in bvFTD and sCJD are disease specific and possibly related to neuropsychiatric symptoms.

  3. Altered Ca2+ homeostasis induces Calpain-Cathepsin axis activation in sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Llorens, Franc; Thüne, Katrin; Sikorska, Beata; Schmitz, Matthias; Tahir, Waqas; Fernández-Borges, Natalia; Cramm, Maria; Gotzmann, Nadine; Carmona, Margarita; Streichenberger, Nathalie; Michel, Uwe; Zafar, Saima; Schuetz, Anna-Lena; Rajput, Ashish; Andréoletti, Olivier; Bonn, Stefan; Fischer, Andre; Liberski, Pawel P; Torres, Juan Maria; Ferrer, Isidre; Zerr, Inga

    2017-04-27

    Sporadic Creutzfeldt-Jakob disease (sCJD) is the most prevalent form of human prion disease and it is characterized by the presence of neuronal loss, spongiform degeneration, chronic inflammation and the accumulation of misfolded and pathogenic prion protein (PrP Sc ). The molecular mechanisms underlying these alterations are largely unknown, but the presence of intracellular neuronal calcium (Ca 2+ ) overload, a general feature in models of prion diseases, is suggested to play a key role in prion pathogenesis.Here we describe the presence of massive regulation of Ca 2+ responsive genes in sCJD brain tissue, accompanied by two Ca 2+ -dependent processes: endoplasmic reticulum stress and the activation of the cysteine proteases Calpains 1/2. Pathogenic Calpain proteins activation in sCJD is linked to the cleavage of their cellular substrates, impaired autophagy and lysosomal damage, which is partially reversed by Calpain inhibition in a cellular prion model. Additionally, Calpain 1 treatment enhances seeding activity of PrP Sc in a prion conversion assay. Neuronal lysosomal impairment caused by Calpain over activation leads to the release of the lysosomal protease Cathepsin S that in sCJD mainly localises in axons, although massive Cathepsin S overexpression is detected in microglial cells. Alterations in Ca 2+ homeostasis and activation of Calpain-Cathepsin axis already occur at pre-clinical stages of the disease as detected in a humanized sCJD mouse model.Altogether our work indicates that unbalanced Calpain-Cathepsin activation is a relevant contributor to the pathogenesis of sCJD at multiple molecular levels and a potential target for therapeutic intervention.

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

    Directory of Open Access Journals (Sweden)

    Irene López González

    2016-02-01

    Full Text Available 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.

  5. Neuronal antibodies in patients with suspected or confirmed sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Rossi, Meghan; Mead, Simon; Collinge, John; Rudge, Peter; Vincent, Angela

    2015-06-01

    There have been reports of patients with antibodies to neuronal antigens misdiagnosed as sporadic Creutzfeldt-Jakob disease (sCJD). Conversely, low levels of antibodies to neuronal proteins have been reported in patients with sCJD. However, the frequency of misdiagnoses, or of antibodies in patients with subsequently confirmed sCJD, is not clear. We reviewed 256 consecutive cases of sCJD seen in the National Prion Clinic, of whom 150 had sera previously referred for selected antibody tests. Eighty-two available samples were retested for antibodies to N-methyl-d-aspartate receptor (NMDAR), the glycine receptor (GlyR), voltage-gated potassium channel (VGKC)-complex and the associated proteins, leucine-rich glioma inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2). Four of the initial 150 sera referred were positive; two had antibodies to NMDAR, and two to the VGKC-complex, one of which was also positive for GlyR antibodies. Of the 82 sCJD sera retested, one had VGKC-complex antibodies confirming the previous result, two had CASPR2 and GlyR antibodies and one had CASPR2 and NMDAR antibodies; all antibodies were at low levels. Over the same period three patients with autoimmune encephalitis and high VGKC-complex antibodies were initially referred as sCJD. This study indicates that VGKC-complex/LGI1 antibodies. Low titres of neuronal antibodies occur only rarely in suspected patients with sCJD and when present should be interpreted with caution. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  6. Voltage-Gated Potassium Channel Autoimmunity Mimicking Creutzfeldt-Jakob Disease

    Science.gov (United States)

    Geschwind, Michael D.; Tan, K. Meng; Lennon, Vanda A.; Barajas, Ramon F.; Haman, Aissa; Klein, Christopher J.; Josephson, S. Andrew; Pittock, Sean J.

    2009-01-01

    Background Rapidly progressive dementia has a variety of causes, including Creutzfeldt-Jakob disease (CJD) and neuronal voltage-gated potassium channel (VGKC) autoantibody–associated encephalopathy. Objective To describe patients thought initially to have CJD but found subsequently to have immunotherapy-responsive VGKC autoimmunity. Design Observational, prospective case series. Setting Department of Neurology, Mayo Clinic, and the Memory and Aging Center, University of California, San Francisco. Patients A clinical serologic cohort of 15 patients referred for paraneoplastic autoantibody evaluation. Seven patients were evaluated clinically by at least one of us. Clinical information for the remaining patients was obtained by physician interview or medical record review. Main Outcome Measures Clinical features, magnetic resonance imaging abnormalities, electroencephalographic patterns, cerebrospinal fluid analyses, and responses to immunomodulatory therapy. Results All the patients presented subacutely with neurologic manifestations, including rapidly progressive dementia, myoclonus, extrapyramidal dysfunction, visual hallucinations, psychiatric disturbance, and seizures; most (60%) satisfied World Health Organization diagnostic criteria for CJD. Magnetic resonance imaging abnormalities included cerebral cortical diffusion-weighted imaging hyperintensities. Electroencephalographic abnormalities included diffuse slowing, frontal intermittent rhythmic delta activity, and focal epileptogenic activity but not periodic sharp wave complexes. Cerebrospinal fluid 14-3-3 protein or neuron-specific enolase levels were elevated in 5 of 8 patients. Hyponatremia was common (60%). Neoplasia was confirmed histologically in 5 patients (33%) and was suspected in another 5. Most patients’ conditions (92%) improved after immunomodulatory therapy. Conclusions Clinical, radiologic, electrophysiologic, and laboratory findings in VGKC autoantibody–associated encephalopathy may be

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

    Science.gov (United States)

    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.

  8. Creutzfeldt-Jakob disease, Heidenhain variant: case report with MRI (DWI) findings

    International Nuclear Information System (INIS)

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

    2004-01-01

    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

  9. Diagnostic profiles of patients with late-onset Creutzfeldt-Jakob disease differ from those of younger Creutzfeldt-Jakob patients: a historical cohort study using data from the German National Reference Center.

    Science.gov (United States)

    Karch, André; Raddatz, Lena Maria; Ponto, Claudia; Hermann, Peter; Summers, David; Zerr, Inga

    2014-05-01

    In contrast to other neurodegenerative diseases, sporadic Creutzfeldt-Jakob disease (sCJD) is rarely diagnosed in patients older than 75 years. Data describing the characteristics of sCJD in the very old are rare and inconclusive. Therefore, a historical cohort study was designed to evaluate clinical, cerebrospinal fluid (CSF), electroencephalography (EEG), and magnetic resonance imaging (MRI) features of this group. Patients older than 75 years identified via the German surveillance program from 2001 to 2012 (n = 73) were compared to a random subsample of sCJD patients younger than 75 (n = 73) from the same time period using an historical cohort design. Older patients showed a faster disease progression represented by an earlier point of diagnosis and a shorter survival time (p disease, older patients presented slightly more often with dementia (p = 0.127) or dysarthria (p = 0.238), whereas disorders of the extrapyramidal (p = 0.056) and visual system (p = 0.015) were more common in the younger group. Atypical MRI profiles such as MRI lesions restricted to one hemisphere (p Creutzfeldt-Jakob disease cases in patients older than 75 years seems likely due to atypical clinical and radiological presentation. This might contribute to lower sCJD incidence rates in this age group.

  10. Patient with rapidly evolving neurological disease with neuropathological lesions of Creutzfeldt-Jakob disease, Lewy body dementia, chronic subcortical vascular encephalopathy and meningothelial meningioma.

    Science.gov (United States)

    Vita, Maria Gabriella; Tiple, Dorina; Bizzarro, Alessandra; Ladogana, Anna; Colaizzo, Elisa; Capellari, Sabina; Rossi, Marcello; Parchi, Piero; Masullo, Carlo; Pocchiari, Maurizio

    2017-04-01

    We report a case of rapidly evolving neurological disease in a patient with neuropathological lesions of Creutzfeldt-Jakob disease (CJD), Lewy body dementia (LBD), chronic subcortical vascular encephalopathy and meningothelial meningioma. The coexistence of severe multiple pathologies in a single patient strengthens the need to perform accurate clinical differential diagnoses in rapidly progressive dementias. © 2016 Japanese Society of Neuropathology.

  11. 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

  12. 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

  13. Agraphia of Kanji (Chinese characters): an early symptom of sporadic Creutzfeldt-Jakob disease in a Japanese patient: a case report.

    Science.gov (United States)

    Nakamura, Keiko; Sakai, Kenji; Samuraki, Miharu; Nozaki, Ichiro; Notoya, Masako; Yamada, Masahito

    2014-08-06

    Slowly progressive cognitive decline is the most frequent initial manifestation in MM2-cortical-type sporadic Creutzfeldt-Jakob disease. Agraphia has never been noted in patients with this type of sporadic Creutzfeldt-Jakob disease, however, we report the case of a Japanese patient with sporadic Creutzfeldt-Jakob disease in whom agraphia of Kanji was an initial cardinal symptom. A 59-year-old right-handed Japanese woman complained of agraphia of Kanji (Chinese characters) as an initial symptom. A neurological examination revealed mild word-finding difficulty, constructive disturbance, hyperreflexia in her jaw and lower limbs, and bilateral extensor plantar reflexes. An examination of her cerebrospinal fluid revealed increased levels of 14-3-3 and total tau proteins, and abnormal conformation of the proteinase K-resistant prion protein. Diffusion-weighted magnetic resonance imaging showed diffuse hyperintensity in bilateral cerebral cortices. Single-photon emission computed tomography scans revealed hypoperfusion in the left temporal lobe, bilateral parietal and occipital lobes. An analysis of the prion protein gene demonstrated no mutation with homozygous for methionine at the codon 129. We diagnosed our patient with sporadic Creutzfeldt-Jakob disease. Although a histological examination was not performed, it was assumed that our patient could be the MM2-cortical type according to the clinical findings and the elevated levels of 14-3-3 protein in her cerebrospinal fluid. The left posterior inferior temporal area, which was affected in our patient as a hypoperfusion area, is associated with selecting and recalling Kanji characters. Focal signs as an early symptom and hypoperfusion areas in sporadic Creutzfeldt-Jakob disease are critical to recognize initial brain lesions damaged by the proteinase K-resistant prion protein accumulation.

  14. Multitracer study with positron emission tomography in Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Engler, Henry [Uppsala University PET Centre, Uppsala University Hospital, SE 751 85 Uppsala (Sweden); Department of Neurology, Uppsala University Hospital, Uppsala (Sweden); Lundberg, Per Olov [Department of Neurology, Uppsala University Hospital, Uppsala (Sweden); Ekbom, Karl [Department of Neurology, Huddinge University Hospital, Stockholm (Sweden); Nennesmo, Inger [Department of Pathology, Huddinge University Hospital, Stockholm (Sweden); Nilsson, Anna; Bergstroem, Mats; Hartvig, Per; Laangstroem, Bengt [Uppsala University PET Centre, Uppsala University Hospital, SE 751 85 Uppsala (Sweden); Tsukada, Hideo [Hamamatsu Photonics K.K.Central Research Lab, Hamakita City (Japan)

    2003-01-01

    During the period February 1997 to April 2000, 15 patients with clinical symptoms of Creutzfeldt-Jakob disease (CJD) were referred to Uppsala University PET Centre. Positron emission tomography (PET) was performed to detect characteristic signs of the disease, e.g. neuronal death and/or astrocytosis in the brain. The examinations were performed in one session starting with oxygen-15 labelled water scan to measure regional cerebral blood flow, followed by imaging with the monoamine oxidase B inhibitor N-[{sup 11}C-methyl]-L-deuterodeprenyl (DED) to assess astrocytosis in the brain and finally imaging with fluorine-18 2-fluorodeoxyglucose (FDG) to assess regional cerebral glucose metabolism (rCMR{sub glu}). Nine of the patients fulfilled the clinical criteria of probable CJD. In eight of them, FDG and DED imaging revealed, in comparison with normal controls, a typical pattern characterized by a pronounced regional decrease (<2SD) in glucose brain metabolism, indicative of neuronal dysfunction; this was accompanied by a similar increase (>2SD) in DED binding, indicating astrocytosis. These changes were most pronounced in the cerebellum and the frontal, occipital and parietal cortices, whereas the pons, the thalamus and the putamen were less affected and the temporal cortex appeared unaffected. The cerebral blood flow showed a pattern similar to that observed with FDG. In the ninth patient, analysis with DED was not possible. The diagnosis of definite CJD according to international consensus criteria was confirmed in six of these patients. In one patient with probable CJD, protease-resistant prion protein (PrPres) could not be demonstrated. In two patients with probable CJD, autopsy was not allowed. Computed tomography and magnetic resonance imaging, performed in four and seven of these nine patients respectively, showed unspecific, mainly atrophic changes. In six other patients, the PET examinations gave a different pattern. In three of them, high rCMR{sub glu} was

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

    Science.gov (United States)

    Grau-Rivera, Oriol; Sánchez-Valle, Raquel; Saiz, Albert; Molinuevo, José Luis; Bernabé, Reyes; Munteis, Elvira; Pujadas, Francesc; Salvador, Antoni; Saura, Júlia; Ugarte, Antonio; Titulaer, Maarten; Dalmau, Josep; Graus, Francesc

    2014-01-01

    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. To determine the frequency of NSA-abs in the cerebrospinal fluid of patients with suspected CJD and in patients with pathologically confirmed (ie, definite) CJD. A mixed prospective (suspected) and retrospective (definite) CJD cohort study was conducted in a reference center for detection of NSA-abs. The population included 346 patients with suspected CJD and 49 patients with definite CJD. Analysis of NSA-abs in cerebrospinal fluid with brain immunohistochemistry optimized for cell-surface antigens was performed. Positive cases in the suspected CJD group were further studied for antigen specificity using cell-based assays. All definite CJD cases were comprehensively tested for NSA-abs, with cell-based assays used for leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein-like 2 (CASPR2), N-methyl-d-aspartate (NMDA), and glycine (GlY) receptors. Neuronal surface antigens were detected in 6 of 346 patients (1.7%) with rapid neurologic deterioration suggestive of CJD. None of these 6 patients fulfilled the diagnostic criteria for probable or possible CJD. The target antigens included CASPR2, LGI1, NMDAR, aquaporin 4, Tr (DNER [δ/notch-like epidermal growth factor-related receptor]), and an unknown protein. Four of the patients developed rapidly progressive dementia, and the other 2 patients had cerebellar ataxia or seizures that were initially considered to be myoclonus without cognitive decline. The patient with Tr-abs had a positive 14-3-3 test result. Small cell lung carcinoma was diagnosed in the patient with antibodies against an unknown antigen. All patients improved or stabilized after appropriate treatment. None of the 49 patients with definite CJD had NSA-abs. A low, but

  16. 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.

    2015-01-01

    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. PMID:25918841

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

    Directory of Open Access Journals (Sweden)

    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

  18. Distinct pathological phenotypes of Creutzfeldt-Jakob disease in recipients of prion-contaminated growth hormone.

    Science.gov (United States)

    Cali, Ignazio; Miller, Cathleen J; Parisi, Joseph E; Geschwind, Michael D; Gambetti, Pierluigi; Schonberger, Lawrence B

    2015-06-25

    The present study compares the clinical, pathological and molecular features of a United States (US) case of growth hormone (GH)-associated Creutzfeldt-Jakob disease (GH-CJD) (index case) to those of two earlier referred US cases of GH-CJD and one case of dura mater (d)-associated CJD (dCJD). All iatrogenic CJD (iCJD) subjects were methionine (M) homozygous at codon 129 (129MM) of the prion protein (PrP) gene and had scrapie prion protein (PrP(Sc)) type 1 (iCJDMM1). The index subject presented with ataxia, weight loss and changes in the sleep pattern about 38 years after the midpoint of GH treatment. Autopsy examination revealed a neuropathological phenotype reminiscent of both sCJDMV2-K (a sporadic CJD subtype in subjects methionine/valine heterozygous at codon 129 with PrP(Sc) type 2 and the presence of kuru plaques) and variant CJD (vCJD). The two earlier cases of GH-CJDMM1 and the one of dCJDMM1 were associated with neuropathological phenotypes that differed from that of the index case mainly because they lacked PrP plaques. The phenotype of the earlier GH-CJDMM1 cases shared several, but not all, characteristics with sCJDMM1, whereas dCJDMM1 was phenotypically indistinguishable from sCJDMM1. Two distinct groups of dCJDMM1 have also been described in Japan based on clinical features, the presence or absence of PrP plaques and distinct PK-resistant PrP(Sc) (resPrP(Sc)) electrophoretic mobilities. The resPrP(Sc) electrophoretic mobility was, however, identical in our GH-CJDMM1 and dCJDMM1 cases, and matched that of sCJDMM1. Our study shows that receipt of prion-contaminated GH can lead to a prion disease with molecular features (129MM and PrP(Sc) type 2) and phenotypic characteristics that differ from those of sporadic prion disease (sCJDMM1), a difference that may reflect adaptation of "heterologous" prion strains to the 129MM background.

  19. Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study.

    Science.gov (United States)

    Cali, Ignazio; Cohen, Mark L; Haїk, Stéphane; Parchi, Piero; Giaccone, Giorgio; Collins, Steven J; Kofskey, Diane; Wang, Han; McLean, Catriona A; Brandel, Jean-Philippe; Privat, Nicolas; Sazdovitch, Véronique; Duyckaerts, Charles; Kitamoto, Tetsuyuki; Belay, Ermias D; Maddox, Ryan A; Tagliavini, Fabrizio; Pocchiari, Maurizio; Leschek, Ellen; Appleby, Brian S; Safar, Jiri G; Schonberger, Lawrence B; Gambetti, Pierluigi

    2018-01-08

    The presence of pathology related to the deposition of amyloid-β (Aβ) has been recently reported in iatrogenic Creutzfeldt-Jakob disease (iCJD) acquired from inoculation of growth hormone (GH) extracted from human cadaveric pituitary gland or use of cadaveric dura mater (DM) grafts.To investigate this phenomenon further, a cohort of 27 iCJD cases - 21 with adequate number of histopathological sections - originating from Australia, France, Italy, and the Unites States, were examined by immunohistochemistry, amyloid staining, and Western blot analysis of the scrapie prion protein (PrP Sc ), and compared with age-group matched cases of sporadic CJD (sCJD), Alzheimer disease (AD) or free of neurodegenerative diseases (non-ND).Cases of iCJD and sCJD shared similar profiles of proteinase K-resistant PrP Sc with the exception of iCJD harboring the "MMi" phenotype. Cerebral amyloid angiopathy (CAA), either associated with, or free of, Thioflavin S-positive amyloid core plaques (CP), was observed in 52% of 21 cases of iCJD, which comprised 37.5% and 61.5% of the cases of GH- and DM-iCJD, respectively. If only cases younger than 54 years were considered, Aβ pathology affected 41%, 2% and 0% of iCJD, sCJD and non-ND, respectively. Despite the patients' younger age CAA was more severe in iCJD than sCJD, while Aβ diffuse plaques, in absence of Aβ CP, populated one third of sCJD. Aβ pathology was by far most severe in AD. Tau pathology was scanty in iCJD and sCJD.In conclusion, (i) despite the divergences in the use of cadaveric GH and DM products, our cases combined with previous studies showed remarkably similar iCJD and Aβ phenotypes indicating that the occurrence of Aβ pathology in iCJD is a widespread phenomenon, (ii) CAA emerges as the hallmark of the Aβ phenotype in iCJD since it is observed in nearly 90% of all iCJD with Aβ pathology reported to date including ours, and it is shared by GH- and DM-iCJD, (iii) although the contributions to Aβ pathology of other

  20. Multitracer study with positron emission tomography in Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Engler, Henry; Lundberg, Per Olov; Ekbom, Karl; Nennesmo, Inger; Nilsson, Anna; Bergstroem, Mats; Hartvig, Per; Laangstroem, Bengt; Tsukada, Hideo

    2003-01-01

    During the period February 1997 to April 2000, 15 patients with clinical symptoms of Creutzfeldt-Jakob disease (CJD) were referred to Uppsala University PET Centre. Positron emission tomography (PET) was performed to detect characteristic signs of the disease, e.g. neuronal death and/or astrocytosis in the brain. The examinations were performed in one session starting with oxygen-15 labelled water scan to measure regional cerebral blood flow, followed by imaging with the monoamine oxidase B inhibitor N-[ 11 C-methyl]-L-deuterodeprenyl (DED) to assess astrocytosis in the brain and finally imaging with fluorine-18 2-fluorodeoxyglucose (FDG) to assess regional cerebral glucose metabolism (rCMR glu ). Nine of the patients fulfilled the clinical criteria of probable CJD. In eight of them, FDG and DED imaging revealed, in comparison with normal controls, a typical pattern characterized by a pronounced regional decrease ( 2SD) in DED binding, indicating astrocytosis. These changes were most pronounced in the cerebellum and the frontal, occipital and parietal cortices, whereas the pons, the thalamus and the putamen were less affected and the temporal cortex appeared unaffected. The cerebral blood flow showed a pattern similar to that observed with FDG. In the ninth patient, analysis with DED was not possible. The diagnosis of definite CJD according to international consensus criteria was confirmed in six of these patients. In one patient with probable CJD, protease-resistant prion protein (PrPres) could not be demonstrated. In two patients with probable CJD, autopsy was not allowed. Computed tomography and magnetic resonance imaging, performed in four and seven of these nine patients respectively, showed unspecific, mainly atrophic changes. In six other patients, the PET examinations gave a different pattern. In three of them, high rCMR glu was noticed in parts of the brain, particularly in the temporal lobes and basal ganglia, which could suggest encephalitis. One of the

  1. Bitemporal hypometabolism in Creutzfeldt-Jakob disease measured by positron emission tomography with [18F]-2-fluorodeoxyglucose

    International Nuclear Information System (INIS)

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

    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 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

  2. High signal of the striatum in sporadic Creutzfeldt-Jakob disease: sequential change on T2-weighted MRI

    International Nuclear Information System (INIS)

    Uemura, A.; O'uchi, T.; Sakamoto, T.; Yashiro, N.

    2002-01-01

    The object of this study is to describe the sequential change of high signal of the striatum on T2-weighted MRI in sporadic Creutzfeldt-Jakob disease (CJD). Three cases of autopsy-proven sporadic CJD and a total of 18 serial MR images are included in this study. The degree of high signal of the striatum on T2-weighted MRI was evaluated by two neuroradiologists and divided into four grades by mutual agreement. Initial MRI of all three cases showed a slightly high signal of the bilateral striatum, and the conspicuity of the high signal became more prominent as the disease progressed. In each case the pathological change of striatum and globus pallidus was compared with the high signal on the last MR image. (orig.)

  3. Distribution and Quantitative Estimates of Variant Creutzfeldt-Jakob Disease Prions in Tissues of Clinical and Asymptomatic Patients.

    Science.gov (United States)

    Douet, Jean Y; Lacroux, Caroline; Aron, Naima; Head, Mark W; Lugan, Séverine; Tillier, Cécile; Huor, Alvina; Cassard, Hervé; Arnold, Mark; Beringue, Vincent; Ironside, James W; Andréoletti, Olivier

    2017-06-01

    In the United-Kingdom, ≈1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities. In this study, we used in vitro amplification of prions by protein misfolding cyclic amplification (PMCA) to estimate distribution and level of the vCJD agent in 21 tissues from 4 patients who died of clinical vCJD and from 1 asymptomatic person with vCJD. PMCA identified major levels of vCJD prions in a range of tissues, including liver, salivary gland, kidney, lung, and bone marrow. Bioassays confirmed that the quantitative estimate of levels of vCJD prion accumulation provided by PMCA are indicative of vCJD infectivity levels in tissues. Findings provide critical data for the design of measures to minimize risk for iatrogenic transmission of vCJD.

  4. High signal of the striatum in sporadic Creutzfeldt-Jakob disease: sequential change on T2-weighted MRI

    Energy Technology Data Exchange (ETDEWEB)

    Uemura, A.; O' uchi, T.; Sakamoto, T.; Yashiro, N. [Department of Radiology, Kameda Medical Center, Kamogawa, Chiba (Japan)

    2002-04-01

    The object of this study is to describe the sequential change of high signal of the striatum on T2-weighted MRI in sporadic Creutzfeldt-Jakob disease (CJD). Three cases of autopsy-proven sporadic CJD and a total of 18 serial MR images are included in this study. The degree of high signal of the striatum on T2-weighted MRI was evaluated by two neuroradiologists and divided into four grades by mutual agreement. Initial MRI of all three cases showed a slightly high signal of the bilateral striatum, and the conspicuity of the high signal became more prominent as the disease progressed. In each case the pathological change of striatum and globus pallidus was compared with the high signal on the last MR image. (orig.)

  5. Diagnosing Sporadic Creutzfeldt-Jakob Disease in a Patient with a Suspected Status Epilepticus in the Intensive Care Unit

    Directory of Open Access Journals (Sweden)

    Harm J. van der Horn

    2013-01-01

    Full Text Available Objective. Several tests are available in the diagnostics of sporadic Creutzfeldt-Jakob disease (sCJD; however, none of these is conclusive. We review the values of these tests, from an intensive care unit (ICU perspective. Methods. Case report and review of the literature. Results. A 53-year-old woman initially presenting with psychiatric symptoms developed myoclonus and was admitted 1 month later to the ICU with a suspected nonconvulsive status epilepticus and respiratory insufficiency, probably due to extensive antiepileptic drug therapy. Typical MRI and EEG findings and a positive 14-3-3 protein led to the diagnosis of sCJD. All treatments were terminated, and autopsy confirmed sCJD. Conclusions. Clinical signs combined with MRI, EEG, and 14-3-3 and/or tau protein determination might be sufficient to diagnose or exclude sCJD and may therefore prevent the application of unnecessary diagnostic tests.

  6. CSF tau correlates with the degree of cortical involvement in E200K familial Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Cohen, Oren S; Chapman, Joab; Korczyn, Amos D; Siaw, Oliver L; Warman-Alaluf, Naama; Nitsan, Zeev; Appel, Shmuel; Kahana, Esther; Rosenmann, Hanna; Hoffmann, Chen

    2016-11-10

    Cerebrospinal fluid (CSF) tau was found to correlate with disease severity and cognitive status in E200K familial Creutzfeldt-Jakob disease (fCJD) patients. The objective of the present study was to test whether tau levels in the CSF also correlate with the disease burden as reflected by the degree of cortical involvement in DWI MRI. Forty-four consecutive E200K fCJD patients (25 males, mean age 58.6±7.5, range 48-75 years) were recruited to the study and had a CSF tau examination as well as measurements of the extent of the cortical involvement in the DWI axial MRI. Correlation was tested using Pearson test. A significant correlation (r=0.617 pdisease burden reinforce the notion that tau can be used as a biomarker reflecting the extent of disease in patients with E200K fCJD. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  7. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  9. [Based on the incidence of Creutzfeldt-Jakob disease in the Lanzarote healthcare area. Description of two definitive cases].

    Science.gov (United States)

    Hernández-Ramos, F J; Martínez Martín, M; Esteban Robayna, M; Jensen Toll, F; Palacios Llopis, S

    2005-01-01

    We present two cases who have been diagnosed of definitive Creutzfeldt-Jakob disease in the health area of Lanzarote in the period January 2002 to January 2004. The two cases are presented with clinical description, complementary tests -- including electroencephalogram, 14-3-3 protein determination -- study of the prionic protein gene, and histopathologic findings. In this article, we try to show the importance of trying to reach a definitive diagnosis with the histopathologic study once there is clinical suspicion (a diagnosis that is probable or possible). In addition our cases show that communication between the clinical and the epidemiological coordinator of the regional community and the National Center of Epidemiology is very important. We refer to the clear growth in the incidence of the disease in the population of Lanzarote in the period above mentioned. Finally, we discuss whether this growth is or is not an isolated event.

  10. Risk of transmission of Creutzfeldt-Jakob disease via blood and blood products. The French risk-analysis over the last 15 years.

    Science.gov (United States)

    Martin, M; Trouvin, J-H

    2013-09-01

    Risk of transmission of Creutzfeldt-Jakob disease (infectious agent, responsible of spongiform encephalopathy) via blood and blood components (including the plasma-derived medicinal products such as coagulation factors and immunoglobulins) have been a subject of concern for Health authorities since the early 1980s, with a regain of interest in the 1990s, with the bovine spongiform encephalopathy outbreak followed few years after with the notification of the first cases of variant Creutzfeldt-Jakob disease in humans. The risk-analysis and measures taken by the French authorities in the period 1990-2010 will be described with the various assumptions and working hypothesis used and revisited as new findings become available. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  11. Biochemical features of genetic Creutzfeldt-Jakob disease with valine-to-isoleucine substitution at codon 180 on the prion protein gene.

    Science.gov (United States)

    Ito, Yoko; Sanjo, Nobuo; Hizume, Masaki; Kobayashi, Atsushi; Ohgami, Tetsuya; Satoh, Katsuya; Hamaguchi, Tsuyoshi; Yamada, Masahito; Kitamoto, Tetsuyuki; Mizusawa, Hidehiro; Yokota, Takanori

    2018-02-19

    Valine-to-isoleucine substitution at codon 180 of the prion protein gene is only observed in patients with Creutzfeldt-Jakob disease and accounts for approximately half of all cases of genetic prion disease in Japan. In the present study, we investigated the biochemical characteristics of valine-to-isoleucine substitution at codon 180 in the prion protein gene, using samples obtained from the autopsied brains of seven patients with genetic Creutzfeldt-Jakob disease exhibiting this mutation (diagnoses confirmed via neuropathological examination). Among these patients, we observed an absence of diglycosylated and monoglycosylated forms of PrP res at codon 181. Our findings further indicated that the abnormal prion proteins were composed of at least three components, although smaller carboxyl-terminal fragments were predominant. Western blot analyses revealed large amounts of PrP res in the cerebral neocortices, where neuropathological examination revealed marked spongiosis. Relatively smaller amounts of PrP res were detected in the hippocampus, where milder spongiosis was observed, than in the cerebral neocortex. These findings indicate that abnormal prion proteins in the neocortex are associated with severe toxicity, resulting in severe spongiosis. Our findings further indicate that the valine-to-isoleucine substitution is not a polymorphism, but rather an authentic pathogenic mutation associated with specific biochemical characteristics that differ from those observed in sporadic Creutzfeldt-Jakob disease. Copyright © 2018 Elsevier Inc. All rights reserved.

  12. Guinea Pig Prion Protein Supports Rapid Propagation of Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease Prions.

    Science.gov (United States)

    Watts, Joel C; Giles, Kurt; Saltzberg, Daniel J; Dugger, Brittany N; Patel, Smita; Oehler, Abby; Bhardwaj, Sumita; Sali, Andrej; Prusiner, Stanley B

    2016-11-01

    The biochemical and neuropathological properties of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) prions are faithfully maintained upon transmission to guinea pigs. However, primary and secondary transmissions of BSE and vCJD in guinea pigs result in long incubation periods of ∼450 and ∼350 days, respectively. To determine if the incubation periods of BSE and vCJD prions could be shortened, we generated transgenic (Tg) mice expressing guinea pig prion protein (GPPrP). Inoculation of Tg(GPPrP) mice with BSE and vCJD prions resulted in mean incubation periods of 210 and 199 days, respectively, which shortened to 137 and 122 days upon serial transmission. In contrast, three different isolates of sporadic CJD prions failed to transmit disease to Tg(GPPrP) mice. Many of the strain-specified biochemical and neuropathological properties of BSE and vCJD prions, including the presence of type 2 protease-resistant PrP Sc , were preserved upon propagation in Tg(GPPrP) mice. Structural modeling revealed that two residues near the N-terminal region of α-helix 1 in GPPrP might mediate its susceptibility to BSE and vCJD prions. Our results demonstrate that expression of GPPrP in Tg mice supports the rapid propagation of BSE and vCJD prions and suggest that Tg(GPPrP) mice may serve as a useful paradigm for bioassaying these prion isolates. Variant Creutzfeldt-Jakob disease (vCJD) and bovine spongiform encephalopathy (BSE) prions are two of the prion strains most relevant to human health. However, propagating these strains in mice expressing human or bovine prion protein has been difficult because of prolonged incubation periods or inefficient transmission. Here, we show that transgenic mice expressing guinea pig prion protein are fully susceptible to vCJD and BSE prions but not to sporadic CJD prions. Our results suggest that the guinea pig prion protein is a better, more rapid substrate than either bovine or human prion protein for

  13. Characteristics of Korean patients with suspected Creutzfeldt-Jakob disease with 14-3-3 protein in cerebrospinal fluid: Preliminary study of the Korean Creutzfeldt-Jakob disease active surveillance program.

    Science.gov (United States)

    Lim, Jae-Sung; Kwon, Hyung-Min; Jang, Jae-Won; Ju, Young-Ran; Kim, SuYeon; Park, Young Ho; Park, So Young; Kim, SangYun

    2015-01-01

    Although Korea had a national surveillance system for Creutzfeldt-Jakob disease (CJD), it was mainly dependent on attending physician's reports. Thus, little prospective data about the epidemiology, characteristics, and final diagnoses of suspected patients were available. We have established a nationwide network for the active surveillance of patients with suspected CJD. When the requested cerebrospinal fluid (CSF) samples tested positive for 14-3-3 protein, we investigated the clinical characteristics of the corresponding patients and followed them until their final diagnoses were confirmed. A total of 218 samples were requested for CSF assays from May 2010 to August 2012, and 106 (48.6%) were positive for 14-3-3 protein. In 89 patients with complete clinical data, 38 (42.7%) were diagnosed with probable CJD and the estimated annual occurrence of CJD was 16.3 persons-per-year. The most common diagnoses of the remainder were central nervous system infection and any-cause encephalopathy. Non-CJD subjects showed worse initial consciousness levels than CJD patients. This preliminary study showed that the number of reported cases of CJD and the true positivity rates of CSF 14-3-3 protein assays were both low in Korea. An active surveillance system is urgently needed to provide the latest nationwide epidemiological data of CJD.

  14. Altered Mitochondria, Protein Synthesis Machinery, and Purine Metabolism Are Molecular Contributors to the Pathogenesis of Creutzfeldt-Jakob Disease.

    Science.gov (United States)

    Ansoleaga, Belén; Garcia-Esparcia, Paula; Llorens, Franc; Hernández-Ortega, Karina; Carmona Tech, Margarita; Antonio Del Rio, José; Zerr, Inga; Ferrer, Isidro

    2016-06-12

    Neuron loss, synaptic decline, and spongiform change are the hallmarks of sporadic Creutzfeldt-Jakob disease (sCJD), and may be related to deficiencies in mitochondria, energy metabolism, and protein synthesis. To investigate these relationships, we determined the expression levels of genes encoding subunits of the 5 protein complexes of the electron transport chain, proteins involved in energy metabolism, nucleolar and ribosomal proteins, and enzymes of purine metabolism in frontal cortex samples from 15 cases of sCJD MM1 and age-matched controls. We also assessed the protein expression levels of subunits of the respiratory chain, initiation and elongation translation factors of protein synthesis, and localization of selected mitochondrial components. We identified marked, generalized alterations of mRNA and protein expression of most subunits of all 5 mitochondrial respiratory chain complexes in sCJD cases. Expression of molecules involved in protein synthesis and purine metabolism were also altered in sCJD. These findings point to altered mRNA and protein expression of components of mitochondria, protein synthesis machinery, and purine metabolism as components of the pathogenesis of CJD. © 2016 American Association of Neuropathologists, Inc. All rights reserved.

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

    Science.gov (United States)

    Iacono, Diego; Ferrari, Sergio; Gelati, Matteo; Zanusso, Gianluigi; Mariotto, Sara; Monaco, Salvatore

    2015-01-01

    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.

  16. Protective Effect of Val129-PrP against Bovine Spongiform Encephalopathy but not Variant Creutzfeldt-Jakob Disease.

    Science.gov (United States)

    Fernández-Borges, Natalia; Espinosa, Juan Carlos; Marín-Moreno, Alba; Aguilar-Calvo, Patricia; Asante, Emmanuel A; Kitamoto, Tetsuyuki; Mohri, Shirou; Andréoletti, Olivier; Torres, Juan María

    2017-09-01

    Bovine spongiform encephalopathy (BSE) is the only known zoonotic prion that causes variant Creutzfeldt-Jakob disease (vCJD) in humans. The major risk determinant for this disease is the polymorphic codon 129 of the human prion protein (Hu-PrP), where either methionine (Met 129 ) or valine (Val 129 ) can be encoded. To date, all clinical and neuropathologically confirmed vCJD cases have been Met 129 homozygous, with the exception of 1 recently reported Met/Val heterozygous case. Here, we found that transgenic mice homozygous for Val 129 Hu-PrP show severely restricted propagation of the BSE prion strain, but this constraint can be partially overcome by adaptation of the BSE agent to the Met 129 Hu-PrP. In addition, the transmission of vCJD to transgenic mice homozygous for Val 129 Hu-PrP resulted in a prion with distinct strain features. These observations may indicate increased risk for vCJD secondary transmission in Val 129 Hu-PrP-positive humans with the emergence of new strain features.

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

    International Nuclear Information System (INIS)

    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

  18. Variants of PLCXD3 are not associated with variant or sporadic Creutzfeldt-Jakob disease in a large international study.

    Science.gov (United States)

    Balendra, Rubika; Uphill, James; Collinson, Claire; Druyeh, Ronald; Adamson, Gary; Hummerich, Holger; Zerr, Inga; Gambetti, Pierluigi; Collinge, John; Mead, Simon

    2016-04-07

    Human prion diseases are relentlessly progressive neurodegenerative disorders which include sporadic Creutzfeldt-Jakob disease (sCJD) and variant CJD (vCJD). Aside from variants of the prion protein gene (PRNP) replicated association at genome-wide levels of significance has proven elusive. A recent association study identified variants in or near to the PLCXD3 gene locus as strong disease risk factors in multiple human prion diseases. This study claimed the first non-PRNP locus to be highly significantly associated with prion disease in genomic studies. A sub-study of a genome-wide association study with imputation aiming to replicate the finding at PLCXD3 including 129 vCJD and 2500 sCJD samples. Whole exome sequencing to identify rare coding variants of PLCXD3. Imputation of relevant polymorphisms was accurate based on wet genotyping of a sample. We found no supportive evidence that PLCXD3 variants are associated with disease. The marked discordance in vCJD genotype frequencies between studies, despite extensive overlap in vCJD cases, and the finding of Hardy-Weinberg disequilibrium in the original study, suggests possible reasons for the discrepancies between studies.

  19. An alarming presentation of Creutzfeldt-Jakob disease following a self-inflicted gunshot wound to the head.

    Science.gov (United States)

    Harnish, Carissa; Gross, Brian; Rittenhouse, Katelyn; Bupp, Katherine; Vellucci, Ashley; Anderson, Jeffrey; Riley, Deborah; Rogers, Frederick B

    2015-05-01

    Transmissible spongiform encephalopathies (TSE), also known as prion diseases, are characterized by rapid and fatal neurological decline. They not only detrimentally affect the patient, but also present additional challenges to healthcare systems due to the infectivity of the tissues and the difficulty of inactivating the prion. The most common TSE is Creutzfeldt-Jakob disease (CJD), which can occur after familial, spontaneous or acquired transmission. TSEs received more attention after the development of variant CJD (vCJD), also known as Mad Cow Disease, in the UK during the mid-1990s. Unlike familial or spontaneous CJD, this variant was connected to consumption of cattle contaminated with the prion disease, bovine spongiform encephalopathy.This development increased interest in the etiology of CJD and other TSEs and the risk it presents as an infectious disease. The following details the case of a 59-year-old male infected with CJD presented to our level II trauma center for treatment following a self-inflicted gunshot wound to the head. Copyright © 2014 Elsevier Ltd. All rights reserved.

  20. Transmissible familial Creutzfeldt-Jakob disease associated with five, seven, and eight extra octapeptide coding repeats in the PRNP gene

    Energy Technology Data Exchange (ETDEWEB)

    Goldfarb, L.G.; Brown, P.; McCombie, W.R.; Gibbs, C.J. Jr.; Gajdusek, D.C. (National Inst. of Health, Bethesda, MD (United States)); Goldgaber, D. (State Univ. of New York, Stony Brook (United States)); Swergold, G.D. (National Inst. of Health, Bethesda, MD (United States)); Wills, P.R. (Univ. of Auckland (New Zealand)); Cervenakova, L. (Inst. of Preventive and Clinical Medicine, Bratislava (Czechoslovakia)); Baron, H. (Searle Pharmaceuticals, Paris (France))

    1991-12-01

    The PRNP gene, encoding the amyloid precursor protein that is centrally involved in Creutzfeldt-Jakob disease (CJD), has an unstable region of five variant tandem octapeptide coding repeats between codons 51 and 91. The authors screened a total of 535 individuals for the presence of extra repeats in this region, including patients with sporadic and familial forms of spongiform encephalopathy, members of their families, other neurological and non-neurological patients, and normal controls. They identified three CJD families (in each of which the proband's disease was neuropathologically confirmed and experimentally transmitted to primates) that were heterozygous for alleles with 10, 12, or 13 repeats, some of which had wobble nucleotide substitutions. They also found one individual with 9 repeats and no nucleotide substitutions who had no evidence of neurological disease. These observations, together with data on published British patients with 11 and 14 repeats, strongly suggest that the occurrence of 10 or more octapeptide repeats in the encoded amyloid precursor protein predisposes to CJD.

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

    International Nuclear Information System (INIS)

    Lee, Jeong Eun; Song, Chang Joon; Lee, In Ho; Yu, In Kyu; Choi, See Sung

    2010-01-01

    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

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

    Directory of Open Access Journals (Sweden)

    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. The Clinical Stages of Sporadic Creutzfeldt-Jakob Disease with Met/Met Genotype in Korean Patients.

    Science.gov (United States)

    Park, So Young; Wang, Min Jeong; Jang, Jae-Won; Park, Young Ho; Lim, Jae-Sung; Youn, Young Chul; Kim, Jungeun; Kim, SangYun

    2016-01-01

    Clinical diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) is currently based on changes occurring in the late disease stages, which limits early-stage detection. Therefore, we investigated the disease course from the vague symptomatic to the terminal phase. We retrospectively reviewed 36 sCJD patient records, classifying the disease progression into 4 stages based on clinical manifestations: vague symptomatic, possible CJD, probable CJD and chronic vegetative state. We analyzed findings from diffusion-weighted imaging (DWI), electroencephalography (EEG) and cerebrospinal fluid (CSF) 14-3-3 protein testing performed at each stage. In stage 1, the most distinctive feature was DWI hyperintensities in the neocortex, even with negative CSF 14-3-3 protein and EEG results. In stage 2, DWI hyperintensities in the limbic cortex were more remarkable. CSF 14-3-3 protein testing yielded positive results in >80% of patients; EEG showed sensitivity in disease stage-dependent differences in clinical symptoms and laboratory test results will facilitate early and accurate diagnosis. © 2016 S. Karger AG, Basel.

  4. Isolated visual symptoms at onset in sporadic Creutzfeldt-Jakob disease: the clinical phenotype of the “Heidenhain variant”

    Science.gov (United States)

    Cooper, S A; Murray, K L; Heath, C A; Will, R G; Knight, R S G

    2005-01-01

    Background: The Heidenhain variant of sporadic Creutzfeldt-Jakob disease (sCJD) is commonly understood to represent cases with early, prominent visual complaints. The term is clarified to represent those who present with isolated visual symptoms. This group may pose diagnostic difficulties and often present to ophthalmologists where they may undergo needless invasive procedures. Method: A retrospective review of 594 pathologically proved sCJD cases referred to the UK National CJD Surveillance Unit over a 15 year period to identify Heidenhain cases. Results: 22 cases had isolated visual symptoms at onset with a mean illness duration of 4 months. The mean age at disease onset was 67 years. Most displayed myoclonus, pyramidal signs, and a delay in the onset of dementia for some weeks. 17 (77%) were referred initially to ophthalmology. Two underwent cataract extraction before diagnosis. All tested cases were homozygous for methionine at codon 129 of the prion protein gene. Conclusions: This rare, but clinically distinct, group of patients with sCJD may cause diagnostic difficulties. Because ocular intervention carries with it the risk of onward transmission awareness of this condition among ophthalmologists is important. PMID:16170128

  5. N-isopropyl I-123 p-iodoamphetamine brain scintigraphy with SPECT in Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Momose, Toshimitsu; Kosaka, Noboru; Nishikawa, Junichi; Ohtake, Tohru; Watanabe, Toshiaki; Yoshikawa, Kohki; Iio, Masahiro

    1988-10-01

    Two patients of clinically diagnosed Creutzfeldt-Jakob disease (CJD) were examined with N-Isopropyl I-123 p-Iodoamphetamine (IMP) SPECT, MRI and XCT. Both patient has myoclonus and severe conscious disturbance with periodic synchronized discharge (PSD) on EEG. SPECT images were obtained using GE400AC/T. Regional IMP uptake was determined by calculating the ratio of each cortical regional to cerebellar IMP uptake (cortico-cerebellar ratio: CCR) and compared with that of five normal controls. In both case, CCR was remarkably decreased in all cortical areas, although XCT or MRI shows no abnormality except slight cortical atrophy. It suggests that metabolic and functional changes proceed morphological abnormality seen on XCT or MRI. In one case of CJD, serial studies of SPECT and XCT were performed after three months of interval. CCR in second study was higher than in first study, while XCT revealed remarkable brain atrophy including cerebrum, pons and cerebellum. It can be interpreted as regional differences of disease process. In conclusion, I-123 IMP-SPECT is useful for the earlier detection of lesions in CJD.

  6. 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.

  7. Risk of transmission of sporadic Creutzfeldt-Jakob disease by surgical procedures: systematic reviews and quality of evidence.

    Science.gov (United States)

    López, Fernando J García; Ruiz-Tovar, María; Almazán-Isla, Javier; Alcalde-Cabero, Enrique; Calero, Miguel; de Pedro-Cuesta, Jesús

    2017-10-01

    Sporadic Creutzfeldt-Jakob disease (sCJD) is potentially transmissible to humans. This study aimed to summarise and rate the quality of the evidence of the association between surgery and sCJD. Firstly, we conducted systematic reviews and meta-analyses of case-control studies with major surgical procedures as exposures under study. To assess quality of evidence, we used the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) approach. Secondly, we conducted a systematic review of sCJD case reports after sharing neurosurgical instruments. Thirteen case-control studies met the inclusion criteria for the systematic review of case-control studies. sCJD was positively associated with heart surgery, heart and vascular surgery and eye surgery, negatively associated with tonsillectomy and appendectomy, and not associated with neurosurgery or unspecified major surgery. The overall quality of evidence was rated as very low. A single case-control study with a low risk of bias found a strong association between surgery conducted more than 20 years before disease onset and sCJD. Seven cases were described as potentially transmitted by reused neurosurgical instruments. The association between surgery and sCJD remains uncertain. Measures currently recommended for preventing sCJD transmission should be strongly maintained. Future studies should focus on the potential association between sCJD and surgery undergone a long time previously.

  8. Case report of Lewy body disease mimicking Creutzfeldt-Jakob disease in a 44-year-old man.

    Science.gov (United States)

    Saint-Aubert, Laure; Pariente, Jérémie; Dumas, Herve; Payoux, Pierre; Brandel, Jean-Philippe; Puel, Michèle; Vital, Anne; Guedj, Eric; Lesage, Suzanne; Peoc'h, Katell; Brefel Courbon, Christine; Ory Magne, Fabienne

    2016-07-30

    Few patients are reported with dementia with Lewy bodies before fifty years-old, which may partly reflect the difficulty of accurate diagnosis in young population. We report the case of a 44-year-old male with pathologically confirmed sporadic dementia with Lewy bodies, who did not fulfil the revised clinical criteria for this disease. We document this atypical case with clinical and cognitive evaluation, imaging, biochemistry, genetics and pathology investigations. Creutzfeldt-Jakob disease was first suspected in this patient with no previous medical history, who developed acute and rapid cognitive impairment, L-dopa-non-responsive parkinsonism, and delusion. Positive 14-3-3 protein was initially detected in cerebrospinal fluid and until the late stages of the disease. Severe atrophy with no diffusion hypersignal was found on structural MRI as well as an extensive hypometabolism on (18)F-FDG-PET, in comparison to age-matched healthy volunteers. Genetic investigation found no alpha-synuclein gene mutation. The patient died within 5 years, and post-mortem examination found numerous Lewy bodies and Lewy neurites consistent with pure Lewy body disease. This comprehensively described case illustrates that dementia with Lewy bodies can occur in young patients with atypical clinical presentation. Biochemistry and neuroimaging investigations can sometimes be insufficient to allow accurate diagnostic. More specific markers to support such diagnosis are needed.

  9. Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease (CJD)

    Energy Technology Data Exchange (ETDEWEB)

    Gabizon, R.; Rosenmann, H.; Meiner, Z.; Kahana, I. (Hadassah Univ., Jerusalem (Israel)); Kahana, E. (Barzilai Medical Center, Ashkelon (Israel)); Shugart, Y.; Ott, J. (Columbia Univ., New York, NY (United States)); Prusiner, S.B. (Univ. of California, San Francisco, CA (United States))

    1993-10-01

    The inherited prion diseases are neurodegenerative disorders which are not only genetic but also transmissible. More than a dozen mutations in the prion protein gene that result in nonconservative amino acid substitutions segregate with the inherited prion diseases including familial Creutzfeldt-Jakob disease (CJD). In Israel, the incidence of CJD is about 1 case/10[sup 4] Libyan Jews. A Lys[sub 200] substitution segregates with CJD and is reported here to be genetically linked to CJD with a lod score of >4.8. Some healthy elderly Lys[sub 200] carriers > age 65 years were identified, suggesting the possibility of incomplete penetrance. In contrast, no linkage was found between the development of familial CJD and a polymorphism encoding either Met[sub 129] or Val[sub 129]. All Libyan Jewish CJD patients with the Lys[sub 200] mutation encode a Met[sub 129] on the mutant allele. Homozygosity for Met[sub 129] did not correlate with age at disease onset or the duration of illness. The frequency of the Met[sub 129] allele was higher in the affected pedigrees than in a control population of Libyan Jews. The frequency of the Met[sub 129] and Val[sub 129] alleles in the control Libyan population was similar to that found in the general Caucasian population. The identification of three Libyan Jews homozygous for the Lys[sub 200] mutation suggests frequent intrafamilial marriages, a custom documented by genealogical investigations. 26 refs., 3 figs., 6 tabs.

  10. N-isopropyl I-123 p-iodoamphetamine brain scintigraphy with SPECT in Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Momose, Toshimitsu; Kosaka, Noboru; Nishikawa, Junichi; Ohtake, Tohru; Watanabe, Toshiaki; Yoshikawa, Kohki; Iio, Masahiro

    1988-01-01

    Two patients of clinically diagnosed Creutzfeldt-Jakob disease (CJD) were examined with N-Isopropyl I-123 p-Iodoamphetamine (IMP) SPECT, MRI and XCT. Both patient has myoclonus and severe conscious disturbance with periodic synchronized discharge (PSD) on EEG. SPECT images were obtained using GE400AC/T. Regional IMP uptake was determined by calculating the ratio of each cortical regional to cerebellar IMP uptake [cortico-cerebellar ratio: CCR] and compared with that of five normal controls. In both case, CCR was remarkably decreased in all cortical areas, although XCT or MRI shows no abnormality except slight cortical atrophy. It suggests that metabolic and functional changes proceed morphological abnormality seen on XCT or MRI. In one case of CJD, serial studies of SPECT and XCT were performed after three months of interval. CCR in second study was higher than in first study, while XCT revealed remarkable brain atrophy including cerebrum, pons and cerebellum. It can be interpreted as regional differences of disease process. In conclusion, I-123 IMP-SPECT is useful for the earlier detection of lesions in CJD. (author)

  11. Amyloid- and FDG-PET in sporadic Creutzfeldt-Jakob disease: Correlation with pathological prion protein in neuropathology.

    Science.gov (United States)

    Matías-Guiu, Jordi A; Guerrero-Márquez, Carmen; Cabrera-Martín, María Nieves; Gómez-Pinedo, Ulises; Romeral, María; Mayo, Diego; Porta-Etessam, Jesús; Moreno-Ramos, Teresa; Carreras, José Luis; Matías-Guiu, Jorge

    2017-05-04

    The role of positron emission tomography (PET) in Creutzfeldt-Jakob disease is less defined than in other neurodegenerative diseases. We studied the correlation between the uptake of 18 F-florbetaben and 18 F-fluorodeoxyglucose with pathological prion protein deposition in histopathology in a case. A patient with 80 y old with a rapid neurological deterioration with a confirmed diagnosis of CJD was studied. PET and MRI studies were performed between 13-20 d before the death. A region of interest analysis was performed using Statistical Parametric Mapping. MRI showed atrophy with no other alterations. FDG-PET showed extensive areas of hypometabolism including left frontoparietal lobes as well as bilateral thalamus. Correlation between uptake of 18 F-florbetaben and pathological prion protein deposition was r = 0.786 (p < 0.05). Otherwise, correlation between uptake of 18 F-FDG and pathological prion protein was r = 0.357 (p = 0.385). Immunohistochemistry with β-amyloid did not show amyloid deposition or neuritic plaques. Our study supports the use of FDG-PET in the assessment of CJD. FDG-PET may be especially useful in cases of suspected CJD and negative MRI. Furthermore, this case report provides more evidence about the behavioral of amyloid tracers, and the possibility of a low-affinity binding to other non-amyloid proteins, such as the pathological prion protein, is discussed.

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

    Directory of Open Access Journals (Sweden)

    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.

  13. [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.

  14. Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 are Identical in Transmission Properties.

    Science.gov (United States)

    Kobayashi, Atsushi; Matsuura, Yuichi; Iwaki, Toru; Iwasaki, Yasushi; Yoshida, Mari; Takahashi, Hitoshi; Murayama, Shigeo; Takao, Masaki; Kato, Shinsuke; Yamada, Masahito; Mohri, Shirou; Kitamoto, Tetsuyuki

    2016-01-01

    The genotype (methionine, M or valine, V) at polymorphic codon 129 of the PRNP gene and the type (1 or 2) of abnormal prion protein in the brain are the major determinants of the clinicopathological features of sporadic Creutzfeldt-Jakob disease (CJD), thus providing molecular basis for classification of sporadic CJD, that is, MM1, MM2, MV1, MV2, VV1 or VV2. In addition to these "pure" cases, "mixed" cases presenting mixed neuropathological and biochemical features have also been recognized. The most frequently observed mixed form is the co-occurrence of MM1 and MM2, namely MM1+2. However, it has remained elusive whether MM1+2 could be a causative origin of dura mater graft-associated CJD (dCJD), one of the largest subgroups of iatrogenic CJD. To test this possibility, we performed transmission experiments of MM1+2 prions and a systematic neuropathological examination of dCJD patients in the present study. The transmission properties of the MM1+2 prions were identical to those of MM1 prions because MM2 prions lacked transmissibility. In addition, the neuropathological characteristics of MM2 were totally absent in dCJD patients examined. These results suggest that MM1+2 can be a causative origin of dCJD and causes neuropathological phenotype similar to that of MM1. © 2015 International Society of Neuropathology.

  15. 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.

  16. 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.

  17. Creutzfeldt-Jakob Disease-Like Periodic Sharp Wave Complexes in Voltage-Gated Potassium Channel-Complex Antibodies Encephalitis: A Case Report.

    Science.gov (United States)

    Savard, Martin; Irani, Sarosh R; Guillemette, Annie; Gosselin-Lefebvre, Stéphanie; Geschwind, Michael; Jansen, Gerard H; Gould, Peter V; Laforce, Robert

    2016-02-01

    Voltage-gated potassium channel-complex antibodies (VGKC-cAbs) encephalitis, a treatable autoantibody encephalopathy, has been previously reported to clinically mimic sporadic Creutzfeldt-Jakob disease. Among available clinical clues to distinguish them, periodic sharp wave complexes, a typical finding in sporadic Creutzfeldt-Jakob disease, have never been reported in association with VGKC-cAbs encephalitis. A 76-year-old man was transferred to a tertiary neurology center with a clinical history of 6-month weight loss, cognitive disturbance, and nonspecific generalized weakness. He had two seizures the month before transfer and then evolved to severe encephalopathy, requiring mechanical ventilation. Periodic sharp wave complexes every 1 to 2 seconds over slowed background were found on EEG, and MRI showed cerebellar and bifrontal cortical T2/FLAIR/DWI hypersignal without restricted diffusion on ADC mapping. Pancorporal positron emission tomography scan was negative. An immunotherapy trial did not improve the patient condition. Therefore, he died after life support withdrawal. Brain autopsy revealed mononuclear neocortex infiltrate without significant spongiosis, and the anti-VGKC test showed a seropositivity of 336 pmol/L (normal, 0-31), 3 month after the patient deceased. This is the first reported case of VGKC-cAbs encephalitis associated with periodic sharp wave complexes on EEG, which further confuse the differential diagnosis with sporadic Creutzfeldt-Jakob disease. However, the cortical DWI hypersignal without restriction seems to remain a way to discriminate these two entities appropriately, when present. These clues are of paramount importance because VGKC-cAbs encephalitis is a treatable disease.

  18. 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)

    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

  19. [A case of MM1+2 Creutzfeldt-Jakob disease with a longitudinal study of EEG and MRI].

    Science.gov (United States)

    Katsube, Mizuho; Shiota, Yuri; Harada, Takayuki; Shibata, Hiroshi; Nagai, Atsushi

    2013-11-01

    We report a case of definite MM1 + 2 sporadic Creutzfeldt-Jakob disease (sCJD). A 66-year-old woman was admitted to our hospital with memory disturbance and disorientation for three months. On admission she presented a progressive cognitive insufficiency. Electroencephalography (EEG) revealed a frontal intermittent rhythmical delta activity (FIRDA) and the brain magnetic resonance imaging (MRI) showed high signal intensities in cerebral cortex on diffusion weighted images (DWI). After four months from the onset, she reached the akinetic mutism state followed by myoclonus. Follow up examination revealed that periodic synchronous discharge (PSD) was found in EEG, and DWI revealed enlargement of high signal intensity lesions in cerebral cortex. At seven months from the onset, PSD and high signal intensities of cortex became unclear with disappearance of myoclonus, and brain white matter lesions were evident on MRI. Serial studies of EEG and MRI revealed that PSD generalized from frontal lobe dominant pattern, while high signal intensity lesions of cortex diffusely increased on DWI. At ten months from the onset patient died. Pathological examination in brain showed moderate and diffuse neuronal cell loss and gliosis in cerebral cortex corresponding with DWI changes. The genotype at codon 129 of the prion protein (PrP) was homozygous methionine (MM) and the type of protease-resistant PrP (PrPres) was the mixed type of 1 and 2 in Western blot analysis. It has been rare to analyze the changes of EEG and MRI in the entire stage and to investigate pathological finding in the case of sCJD-MM1 + 2. A longitudinal examination of EEG and MRI is useful for early diagnosis of CJD. Also we could correlate these findings with clinical and histopathological phenotype.

  20. Brains with sporadic Creutzfeldt-Jakob disease and copathology showed a prolonged end-stage of disease.

    Science.gov (United States)

    Miguelez-Rodriguez, Aitzol; Santos-Juanes, Jorge; Vicente-Etxenausia, Ikerne; Perez de Heredia-Goñi, Katty; Garcia, Beatriz; Quiros, Luis M; Lorente-Gea, Laura; Guerra-Merino, Isabel; Aguirre, Jose J; Fernandez-Vega, Ivan

    2018-05-01

    To investigate the expression of major proteins related to primary neurodegenerative diseases and their prognostic significance in brains with Creutzfeldt-Jakob disease (CJD). Thirty consecutive cases of confirmed CJD during the period 2010-2015 at Basque Brain bank were retrospectively reviewed. Moreover, major neurodegenerative-associated proteins (phosphorylated Tau, 4R tau, 3R tau, alpha-synuclein, TDP43, amyloid beta) were tested. Clinical data were reviewed. Cases were divided according to the presence or absence of copathology. Survival curves were also determined. Copathology was significantly associated with survival in brains with CJD (4.2±1.2 vs 9.2±1.9; P=0.019) and in brains with MM1/MV1 CJD (2.1±1.0 vs 6.7±2.8; P=0.012). Besides, the presence of more than one major neurodegenerative-associated protein was significantly associated with survival (4.2±1.2 vs 10.7±2.6; P=0.017). Thus, univariate analyses further pointed out variables significantly associated with better survival: copathology in CJD (HR=0.430; P=0.033); more than one neurodegenerative-associated protein in CJD (HR=0.369; P=0.036) and copathology in MM1/MV1 CJD (HR=0.525; P=0.032). The existence of copathology significantly prolongs survival in patients with rapidly progressive dementia due to CJD. The study of major neurodegenerative-associated proteins in brains with CJD could allow us to further understand the molecular mechanisms behind prion diseases. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  1. Sporadic Creutzfeldt-Jakob disease with unusual initial presentation as posterior reversible encephalopathy syndrome: a case report.

    Science.gov (United States)

    Dirzius, Edgaras; Balnyte, Renata; Steibliene, Vesta; Gleizniene, Rymante; Gudinaviciene, Inga; Radziunas, Andrius; Petrikonis, Kestutis

    2016-11-22

    Creutzfeldt - Jakob disease (CJD) is a rapidly progressive and fatal neurodegenerative prion disease. MRI findings are included in diagnostic criteria for probable CJD, giving a sensitivity and specificity more than 90%, but the atypical radiological presentations in the early stage of the disease could cause the diagnostic difficulties. CJD can be definitively diagnosed by histopathological confirmation, brain biopsy or at autopsy. We present a case of 53-year-old woman with a history of a rapidly progressive dementia with symptoms of visual impairment, increased extrapyramidal type muscle tonus, stereotypical movements and ataxic gait resulting in the patient's death after13 months. The clinical symptoms deteriorated progressively to myoclonus and akinetic mutism already on the 14th week. The series of diagnostic examinations were done to exclude the possible causes of dementia. Initial MRI evaluation as posterior reversible encephalopathy syndrome (PRES) on the 9th week after the onset of symptoms created us a diagnostic conundrum. Subsequent MRI findings of symmetrical lesions in the basal ganglia (nucleus caudatus, putamen) on the 13th week and EEG with periodic sharp wave complexes (PSWC) in frontal regions on the 18th week allowed us to diagnose the probable sCJD. The histopathological findings after brain biopsy on the 14th week demonstrated the presence of the abnormal prion protein deposits in the grey matter by immunohistochemistry with ICSM35, KG9 and 12 F10 antibodies and confirmed the diagnosis of sCJD. In this article we focus our attention on a rare association between radiological PRES syndrome and early clinical stage of sCJD. Although concurrent manifestation of these conditions can be accidental, but the immunogenic or neuropeptide mechanisms could explain such radiological MRI findings. A thorough knowledge of differential diagnostic of PRES may be especially useful in earlier diagnosis of sCJD.

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

    International Nuclear Information System (INIS)

    Kim, Jae Hyoung; Choi, Byung Se; Jung, Cheolkyu; Chang, YoungHee; Kim, SangYun

    2011-01-01

    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.)

  3. Validation of 14-3-3 Protein as a Marker in Sporadic Creutzfeldt-Jakob Disease Diagnostic.

    Science.gov (United States)

    Schmitz, Matthias; Ebert, Elisabeth; Stoeck, Katharina; Karch, André; Collins, Steven; Calero, Miguel; Sklaviadis, Theodor; Laplanche, Jean-Louis; Golanska, Ewa; Baldeiras, Ines; Satoh, Katsuya; Sanchez-Valle, Raquel; Ladogana, Anna; Skinningsrud, Anders; Hammarin, Anna-Lena; Mitrova, Eva; Llorens, Franc; Kim, Yong Sun; Green, Alison; Zerr, Inga

    2016-05-01

    At present, the testing of 14-3-3 protein in cerebrospinal fluid (CSF) is a standard biomarker test in suspected sporadic Creutzfeldt-Jakob disease (sCJD) diagnosis. Increasing 14-3-3 test referrals in CJD reference laboratories in the last years have led to an urgent need to improve established 14-3-3 test methods. The main result of our study was the validation of a commercially available 14-3-3 ELISA next to the commonly used Western blot method as a high-throughput screening test. Hereby, 14-3-3 protein expression was quantitatively analyzed in CSF of 231 sCJD and 2035 control patients. We obtained excellent sensitivity/specificity values of 88 and 96% that are comparable to the established Western blot method. Since standard protocols and preanalytical sample handling have become more important in routine diagnostic, we investigated in a further step the reproducibility and stability of 14-3-3 as a biomarker for human prion diseases. Ring trial data from 2009 to 2013 revealed an increase of Fleiss' kappa from 0.51 to 0.68 indicating an improving reliability of 14-3-3 protein detection. The stability of 14-3-3 protein under short-term and long-term storage conditions at various temperatures and after repeated freezing/thawing cycles was confirmed. Contamination of CSF samples with blood appears likely to be an important factor at a concentration of more than 2500 erythrocytes/μL. Hemolysis of erythrocytes with significant release of 14-3-3 protein started after 2 days at room temperature. We first define clear standards for the sample handling, short- and long-term storage of CSF samples as well as the handling of blood- contaminated samples which may result in artificially elevated CSF levels of 14-3-3.

  4. Revisiting the Heidenhain Variant of Creutzfeldt-Jakob Disease: Evidence for Prion Type Variability Influencing Clinical Course and Laboratory Findings.

    Science.gov (United States)

    Baiardi, Simone; Capellari, Sabina; Ladogana, Anna; Strumia, Silvia; Santangelo, Mario; Pocchiari, Maurizio; Parchi, Piero

    2016-01-01

    The Heidenhain variant defines a peculiar clinical presentation of sporadic Creutzfeldt-Jakob disease (sCJD) characterized by isolated visual disturbances at disease onset and reflecting the early targeting of prions to the occipital cortex. Molecular and histopathological typing, thus far performed in 23 cases, has linked the Heidenhain variant to the MM1 sCJD type. To contribute a comprehensive characterization of cases with the Heidenhain variant, we reviewed a series of 370 definite sCJD cases. Eighteen patients (4.9%) fulfilled the selection criteria. Fourteen of them belonging to sCJD types MM1 or MM1+2C had a short duration of isolated visual symptoms and overall clinical disease, a high prevalence of periodic sharp-wave complexes in EEG, and a marked increase of cerebrospinal fluid proteins t-tau and 14-3-3 levels. In contrast, three cases of the MM 2C or MM 2+1C types showed a longer duration of isolated visual symptoms and overall clinical disease, non-specific EEG findings, and cerebrospinal fluid concentration below threshold for the diagnosis of "probable" CJD of both 14-3-3 and t-tau. However, a brain DWI-MRI disclosed an occipital cortical hyperintensity in the majority of examined cases of both groups. While confirming the strong linkage with the methionine genotype at the polymorphic codon 129 of the prion protein gene, our results definitely establish that the Heidenhain variant can also be associated with the MM 2C sCJD type in addition to the more common MM1 type. Likewise, our results highlight the significant differences in clinical evolution and laboratory findings between cases according to the dominant PrPSc type (type 1 versus type 2).

  5. 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.)

  6. Beskytter røyking mot Parkinsons sykdom

    Directory of Open Access Journals (Sweden)

    Trond Peder Flaten

    2009-10-01

    Full Text Available  SAMMENDRAGDen negative sammenhengen mellom røyking og Parkinsons sykdom i epidemiologiske studier er robust.Det er prosentvis færre røykere og eks-røykere blant Parkinson-pasienter enn blant kontrollpersoner.Videre har røykere en lavere insidens og en lavere dødelighet enn andre av Parkinsons sykdom. Det er etåpent spørsmål om dette skyldes en beskyttende virkning av nikotin eller andre stoffer i tobakksrøyk somhydrazin eller karbonmonoksid, eller om det skyldes andre, underliggende faktorer. De mest aktuelle slikefaktorer er (1 selektiv dødelighet, altså at mange røykere ikke overlever lenge nok til å utvikleParkinsons sykdom, og (2 at personer som utvikler Parkinsons sykdom ofte har karaktertrekk som gjørdet mindre sannsynlig at de begynner å røyke. Det vitenskapelige grunnlaget er i alle fall for tynt for åanbefale røyking som et forebyggende virkemiddel mot Parkinsons sykdom. Og selv om det skulle viseseg at røyking virkelig beskytter mot sykdommen, veier de negative helsevirkningene av røyking myetyngre i folkehelsesammenheng. Allikevel bør denne assosiasjonen forfølges videre, ikke minst forpotensialet for en bedret forståelse av de underliggende patofysiologiske mekanismene bak sykdommen.Flaten TP. Does smoking protect against Parkinson’s disease? Nor J Epidemiol 1995; 5 (2: 145-150. ENGLISH SUMMARYIt is epidemiologically well established that Parkinson's disease is about twice as common among nonsmokers as among smokers. Whether this is due to a protective effect of nicotine or other compounds in cigarette smoke like hydrazine or carbon monoxide, or whether it is due to some other, confounding factor(s, remains to be established. The most relevant confounding factors are (1 selective mortality, that is, many smokers do not survive long enough to develop Parkinson's disease, and (2 that persons who eventually develop Parkinson's disease tend to have a preclinical personality type that makes them less likely

  7. What could be the role of quinacrine in Creutzfeldt-Jakob disease treatment?

    Czech Academy of Sciences Publication Activity Database

    Zawada, Zbigniew; Šebestík, Jaroslav; Šafařík, Martin; Krejčiříková, A.; Hlaváček, Jan; Stibor, Ivan; Holada, K.; Bouř, Petr

    2010-01-01

    Roč. 16, S2 (2010), s. 55-55 ISSN 1075-2617. [European Peptide Symposium /31./. 05.09.2010-09.09.2010, Copenhagen] R&D Projects: GA ČR GA203/07/1517 Institutional research plan: CEZ:AV0Z40550506 Keywords : prion * quinacrine * thiolysis Subject RIV: CC - Organic Chemistry

  8. Reaction of quinacrine with prion protein: treatment for Creutzfeldt-Jakob disease?

    Czech Academy of Sciences Publication Activity Database

    Zawada, Zbigniew; Šebestík, Jaroslav; Šafařík, Martin; Březinová, Anna; Bouř, Petr; Hlaváček, Jan; Stibor, Ivan

    2010-01-01

    Roč. 104, č. 11 (2010), s. 1129-1129 ISSN 0009-2770. [Pokroky v organické, bioorganické a farmaceutické chemii /45./. 20.11.2010-22.11.2010, Nymburk] R&D Projects: GA ČR GA203/07/1517 Institutional research plan: CEZ:AV0Z40550506 Keywords : quinacrine * acridine displacement * prions * prevention of aggregation Subject RIV: CC - Organic Chemistry

  9. Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance

    Science.gov (United States)

    2013-01-01

    Background There is increasing epidemiological evidence of etiological links between general surgery and sporadic Creutzfeldt-Jakob disease (sCJD) with long incubation periods. The purpose of this study was to identify specific surgical procedures potentially associated with sCJD to be targeted for preventive presurgical-intervention guidance. Results We propose a three-step clinical guidance outline where surgical procedures associated with sCJD clinical onset – potentially more contaminant - are taken into account. Data on hospital discharges and surgical procedures were obtained from Danish and Swedish national in-patient hospital registries for 167 sCJD cases, onset 1987–2003, and for 835 matched and 2,224 unmatched population controls. Surgery was allocated to different life-time periods as previously reported, and frequencies were compared using logistic regression analysis. In the year preceding clinical onset, persons with sCJD underwent a statistically significant higher number of minor surgical interventions (OR (95% CI): 17.50 (3.64-84.24)), transluminal endoscopies (OR: 2.73 (1.01–7.37)) and gastrointestinal operations (OR: 3.51 (1.21–10.19)) compared to matched controls. Surgical discharges clustered towards clinical onset. These differences increased during the clinical period, with statistically significant higher frequencies for both endoscopies and minor surgery (OR: 13.91 (5.87-32.95), and for main surgical procedures (OR: 2.10 (1.00-4.39)), particularly gastrointestinal surgery (OR: 6.00 (1.83-19.66)), and surgery contacting skeletal muscle. Comparisons with unmatched controls yielded similar results for neurosurgery in the clinical period (OR: 19.40 (2.22-168.34)). Conclusions These results suggest that some types of surgical procedures are associated with sCJD, after clinical onset or particularly just before onset. Selective planning of such surgery to minimize instrument/device contamination or quarantining might be feasible

  10. 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

    2013-01-01

    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. PMID:23792955

  11. 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.

  12. Evolution of Diffusion-Weighted Magnetic Resonance Imaging Signal Abnormality in Sporadic Creutzfeldt-Jakob Disease, With Histopathological Correlation.

    Science.gov (United States)

    Eisenmenger, Laura; Porter, Marie-Claire; Carswell, Christopher J; Thompson, Andrew; Mead, Simon; Rudge, Peter; Collinge, John; Brandner, Sebastian; Jäger, Hans R; Hyare, Harpreet

    2016-01-01

    Prion diseases represent the archetype of brain diseases caused by protein misfolding, with the most common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia. Diffusion-weighted imaging (DWI) has emerged as the most sensitive magnetic resonance imaging (MRI) sequence for the diagnosis of sCJD, but few studies have assessed the evolution of MRI signal as the disease progresses. To assess the natural history of the MRI signal abnormalities on DWI in sCJD to improve our understanding of the pathogenesis and to investigate the potential of DWI as a biomarker of disease progression, with histopathological correlation. Gray matter involvement on DWI was assessed among 37 patients with sCJD in 26 cortical and 5 subcortical subdivisions per hemisphere using a semiquantitative scoring system of 0 to 2 at baseline and follow-up. A total brain score was calculated as the summed scores in the individual regions. In 7 patients, serial mean diffusivity measurements were obtained. Age at baseline MRI, disease duration, atrophy, codon 129 methionine valine polymorphism, Medical Research Council Rating Scale score, and histopathological findings were documented. The study setting was the National Prion Clinic, London, England. All participants had a probable or definite diagnosis of sCJD and had at least 2 MRI studies performed during the course of their illness. The study dates were October 1, 2008 to April 1, 2012. The dates of our analysis were January 19 to April 20, 2012. Correlation of regional and total brain scores with disease duration. Among the 37 patients with sCJD in this study there was a significant increase in the number of regions demonstrating signal abnormality during the study period, with 59 of 62 regions showing increased signal intensity (SI) at follow-up, most substantially in the caudate and putamen (P disease duration (r = 0.47, P = .003 at baseline and r = 0.35, P = .03 at follow-up), and the left

  13. CSF Tau proteins reduce misdiagnosis of sporadic Creutzfeldt-Jakob disease suspected cases with inconclusive 14-3-3 result.

    Science.gov (United States)

    Leitão, M J; Baldeiras, I; Almeida, M R; Ribeiro, M H; Santos, A C; Ribeiro, M; Tomás, J; Rocha, S; Santana, I; Oliveira, C R

    2016-09-01

    Cerebrospinal fluid (CSF) 14-3-3 protein supports sporadic Creutzfeldt-Jakob (sCJD) diagnosis, but often leads to weak-positive results and lacks standardization. In this study, we explored the added diagnostic value of Total Tau (t-Tau) and phosphorylated Tau (p-Tau) in sCJD diagnosis, particularly in the cases with inconclusive 14-3-3 result. 95 definite sCJD and 287 patients without prion disease (non-CJD) were included in this study. CSF samples were collected in routine clinical diagnosis and analysed for 14-3-3 detection by Western blot (WB). CSF t-Tau and p-Tau were quantified by commercial ELISA kits and PRNP and APOE genotyping assessed by PCR-RFLP. In a regression analysis of the whole cohort, 14-3-3 protein revealed an overall accuracy of 82 % (sensitivity = 96.7 %; specificity = 75.6 %) for sCJD. Regarding 14-3-3 clear positive results, we observed no added value either of t-Tau alone or p-Tau/t-Tau ratio in the model. On the other hand, considering 14-3-3 weak-positive cases, t-Tau protein increased the overall accuracy of 14-3-3 alone from 91 to 94 % and specificity from 74 to 93 % (p < 0.05), with no sensitivity improvement. However, inclusion of p-Tau/t-Tau ratio did not significantly improve the first model (p = 0.0595). Globally, t-Tau protein allowed a further discrimination of 65 % within 14-3-3 inconclusive results. Furthermore, PRNP MV genotype showed a trend to decrease 14-3-3 sensitivity (p = 0.051), but such effect was not seen on t-Tau protein. In light of these results, we suggest that t-Tau protein assay is of significant importance as a second marker in identifying 14-3-3 false-positive results among sCJD probable cases.

  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

    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

  15. Creutzfeldt-Jakob Disease with a prion protein gene codon 180 mutation presenting asymmetric cortical high-intensity on magnetic resonance imaging.

    Science.gov (United States)

    Amano, Yuko; Kimura, Noriyuki; Hanaoka, Takuya; Aso, Yasuhiro; Hirano, Teruyuki; Murai, Hiroyuki; Satoh, Katsuya; Matsubara, Etsuro

    2015-01-01

    Here we report a genetically confirmed case of Creutzfeldt-Jakob disease with a prion protein gene codon 180 mutation presenting atypical magnetic resonance imaging findings. The present case exhibited an acute onset and lateralized neurologic signs, and progressive cognitive impairment. No myoclonus or periodic synchronous discharges on electroencephalography were observed. Diffusion-weighted images revealed areas of high signal intensity in the right frontal and temporal cortices at onset that extended to the whole cortex and basal ganglia of the right cerebral hemisphere at 3 months. Although the cerebrospinal fluid (CSF) was initially negative for neuron specific enolase, tau protein, 14-3-3 protein, and abnormal prion protein, the CSF was positive for these brain-derived proteins at 3 months after onset.

  16. Strain-Specific Altered Regulatory Response of Rab7a and Tau in Creutzfeldt-Jakob Disease and Alzheimer's Disease.

    Science.gov (United States)

    Zafar, Saima; Younas, Neelam; Correia, Susana; Shafiq, Mohsin; Tahir, Waqas; Schmitz, Matthias; Ferrer, Isidre; Andréoletti, Olivier; Zerr, Inga

    2017-01-01

    There is an increasing demand for the understanding of pathophysiology on neurodegeneration diseases at early stages. Changes in endocytic machinery and the cytoskeleton-associated response are the first alterations observed in Creutzfeldt-Jakob disease (CJD) and Alzheimer's disease AD brain. In this study, we performed a targeted search for endocytic pathway proteins in the different regions of the brain. We found late endosome marker Rab7a which was significantly upregulated in the frontal cortex region in the rapid progressive CJD form (MM1) and rapid progressive AD (rpAD) forms. However, Rab9 expression was significantly downregulated only in CJD-MM1 brain frontal cortex region. In the cerebellum, Rab7a expression showed significant upregulation in both subtype MM1 and VV2 CJD forms, in contrast to Rab9 which showed significant downregulation in both subtype MM1 and VV2 CJD forms at terminal stage of the disease. To check regulatory response at pre-symptomatic stage of the disease, we checked the regulatory interactive response of Rab7a, Rab9, and known biomarkers PrP C and tau forms in frontal cortex at pre-symptomatic stage of the disease in tg340 mice expressing about fourfold of human PrP-M129 with PrP-null background that had been inoculated with human sCJD MM1 brain tissue homogenates (sCJD MM1 mice). In addition, we analyzed 5XFAD mice, exhibiting five mutations in the APP and presenilin genes related to familial Alzheimer's disease (FAD), to validate specific regulatory response of Rab7a, Rab9, tau, and phosphorylated form of tau by immunostaining 5XFAD mice in comparison with the wild-type age-matched mice brain. The cortical region of 5XFAD mice brain showed accumulated form of Rab7a in puncta that co-label for p-Tau, indicating colocalization by using confocal laser-scanning microscopy and was confirmed by using reverse co-immunoprecipitation. Furthermore, synthetic RNA (siRNA) against the Rab7a gene decreased expression of Rab7a protein, in cortical

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

    Science.gov (United States)

    2011-01-01

    Background To better characterize the value of cerebrospinal fluid (CSF) proteins as diagnostic markers in a clinical population of subacute encephalopathy patients with relatively low prevalence of sporadic Creutzfeldt-Jakob disease (sCJD), we studied the diagnostic accuracies of several such markers (14-3-3, tau and S100B) in 1000 prospectively and sequentially recruited Canadian patients with clinically suspected sCJD. Methods The study included 127 patients with autopsy-confirmed sCJD (prevalence = 12.7%) and 873 with probable non-CJD diagnoses. Standard statistical measures of diagnostic accuracy were employed, including sensitivity (Se), specificity (Sp), predictive values (PVs), likelihood ratios (LRs), and Receiver Operating Characteristic (ROC) analysis. Results At optimal cutoff thresholds (empirically selected for 14-3-3, assayed by immunoblot; 976 pg/mL for tau and 2.5 ng/mL for S100B, both assayed by ELISA), Se and Sp respectively were 0.88 (95% CI, 0.81-0.93) and 0.72 (0.69-0.75) for 14-3-3; 0.91 (0.84-0.95) and 0.88 (0.85-0.90) for tau; and 0.87 (0.80-0.92) and 0.87 (0.84-0.89) for S100B. The observed differences in Sp between 14-3-3 and either of the other 2 markers were statistically significant. Positive LRs were 3.1 (2.8-3.6) for 14-3-3; 7.4 (6.9-7.8) for tau; and 6.6 (6.1-7.1) for S100B. Negative LRs were 0.16 (0.10-0.26) for 14-3-3; 0.10 (0.06-0.20) for tau; and 0.15 (0.09-0.20) for S100B. Estimates of areas under ROC curves were 0.947 (0.931-0.961) for tau and 0.908 (0.888-0.926) for S100B. Use of interval LRs (iLRs) significantly enhanced accuracy for patient subsets [e.g., 41/120 (34.2%) of tested sCJD patients displayed tau levels > 10,000 pg/mL, with an iLR of 56.4 (22.8-140.0)], as did combining tau and S100B [e.g., for tau > 976 pg/mL and S100B > 2.5 ng/mL, positive LR = 18.0 (12.9-25.0) and negative LR = 0.02 (0.01-0.09)]. Conclusions CSF 14-3-3, tau and S100B proteins are useful diagnostic markers of sCJD even in a low

  18. Creutzfeldt-Jakob Disease

    Science.gov (United States)

    ... CJD diagnosed? Several tests can help diagnose CJD. Electroencephalography (EEG), which records the brain’s electrical pattern, can be ... to be accurate in about 90 percent of cases. The only way to confirm a ... can cure or control CJD, although studies of a variety of drugs are now in ...

  19. Creutzfeldt-Jakob Disease

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    ... in the chromosome 20 gene coding the biological blueprint for prion protein. People who develop familial CJD ... other dementias, and help you find local support services. Call our 24/7 Helpline at 800.272. ...

  20. 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

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    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

  1. Inhibition of IL-1β Signaling Normalizes NMDA-Dependent Neurotransmission and Reduces Seizure Susceptibility in a Mouse Model of Creutzfeldt-Jakob Disease.

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    Bertani, Ilaria; Iori, Valentina; Trusel, Massimo; Maroso, Mattia; Foray, Claudia; Mantovani, Susanna; Tonini, Raffaella; Vezzani, Annamaria; Chiesa, Roberto

    2017-10-25

    Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder caused by prion protein (PrP) misfolding, clinically recognized by cognitive and motor deficits, electroencephalographic abnormalities, and seizures. Its neurophysiological bases are not known. To assess the potential involvement of NMDA receptor (NMDAR) dysfunction, we analyzed NMDA-dependent synaptic plasticity in hippocampal slices from Tg(CJD) mice, which model a genetic form of CJD. Because PrP depletion may result in functional upregulation of NMDARs, we also analyzed PrP knock-out (KO) mice. Long-term potentiation (LTP) at the Schaffer collateral-commissural synapses in the CA1 area of ∼100-d-old Tg(CJD) mice was comparable to that of wild-type (WT) controls, but there was an inversion of metaplasticity, with increased GluN2B phosphorylation, which is indicative of enhanced NMDAR activation. Similar but less marked changes were seen in PrP KO mice. At ∼300 d of age, the magnitude of LTP increased in Tg(CJD) mice but decreased in PrP KO mice, indicating divergent changes in hippocampal synaptic responsiveness. Tg(CJD) but not PrP KO mice were intrinsically more susceptible than WT controls to focal hippocampal seizures induced by kainic acid. IL-1β-positive astrocytes increased in the Tg(CJD) hippocampus, and blocking IL-1 receptor signaling restored normal synaptic responses and reduced seizure susceptibility. These results indicate that alterations in NMDA-dependent glutamatergic transmission in Tg(CJD) mice do not depend solely on PrP functional loss. Moreover, astrocytic IL-1β plays a role in the enhanced synaptic responsiveness and seizure susceptibility, suggesting that targeting IL-1β signaling may offer a novel symptomatic treatment for CJD. SIGNIFICANCE STATEMENT Dementia and myoclonic jerks develop in individuals with Creutzfeldt-Jakob disease (CJD), an incurable brain disorder caused by alterations in prion protein structure. These individuals are prone to seizures and have high

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

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    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.

  3. Accuracy of diagnosis criteria in patients with suspected diagnosis of sporadic Creutzfeldt-Jakob disease and detection of 14-3-3 protein, France, 1992 to 2009.

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    Peckeu, Laurene; Delasnerie-Lauprètre, Nicole; Brandel, Jean-Philippe; Salomon, Dominique; Sazdovitch, Véronique; Laplanche, Jean-Louis; Duyckaerts, Charles; Seilhean, Danielle; Haïk, Stéphane; Hauw, Jean-Jacques

    2017-10-01

    Diagnostic criteria of Creutzfeldt-Jakob disease (CJD), a rare and fatal transmissible nervous system disease with public health implications, are determined by clinical data, electroencephalogram (EEG), detection of 14-3-3 protein in cerebrospinal fluid (CSF), brain magnetic resonance imaging and prion protein gene examination. The specificity of protein 14-3-3 has been questioned. We reviewed data from 1,572 autopsied patients collected over an 18-year period (1992-2009) and assessed whether and how 14-3-3 detection impacted the diagnosis of sporadic CJD in France, and whether this led to the misdiagnosis of treatable disorders. 14-3-3 detection was introduced into diagnostic criteria for CJD in 1998. Diagnostic accuracy decreased from 92% for the 1992-1997 period to 85% for the 1998-2009 period. This was associated with positive detections of 14-3-3 in cases with negative EEG and alternative diagnosis at autopsy. Potentially treatable diseases were found in 163 patients (10.5%). This study confirms the usefulness of the recent modification of diagnosis criteria by the addition of the results of CSF real-time quaking-induced conversion, a method based on prion seed-induced misfolding and aggregation of recombinant prion protein substrate that has proven to be a highly specific test for diagnosis of sporadic CJD.

  4. Detection and Localization of PrPSc in the Skeletal Muscle of Patients with Variant, Iatrogenic, and Sporadic Forms of Creutzfeldt-Jakob Disease

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    Peden, Alexander H.; Ritchie, Diane L.; Head, Mark W.; Ironside, James W.

    2006-01-01

    Variant Creutzfeldt-Jakob disease (vCJD) differs from other human prion diseases in that the pathogenic prion protein PrPSc can be detected to a greater extent at extraneuronal sites throughout the body, principally within lymphoid tissues. However, a recent study using a high-sensitivity Western blotting technique revealed low levels of PrPSc in skeletal muscle from a quarter of Swiss patients with sporadic CJD (sCJD). This posed the question of whether PrPSc in muscle could also be detected in vCJD, sCJD, and iatrogenic (iCJD) patients from other populations. Therefore, we have used the same high-sensitivity Western blotting technique, in combination with paraffin-embedded tissue blotting, to screen for PrPSc in muscle tissue specimens taken at autopsy from 49 CJD patients in the United Kingdom. These techniques identified muscle PrPSc in 8 of 17 vCJD, 7 of 26 sCJD, and 2 of 5 iCJD patients. Paraffin-embedded tissue blotting analysis showed PrPSc in skeletal muscle in localized anatomical structures that had the morphological and immunohistochemical characteristics of nerve fibers. The detection of PrPSc in muscle tissue from all forms of CJD indicates the possible presence of infectivity in these tissues, suggesting important implications for assessing the potential risk of iatrogenic spread via contaminated surgical instruments. PMID:16507908

  5. UK Iatrogenic Creutzfeldt-Jakob disease: investigating human prion transmission across genotypic barriers using human tissue-based and molecular approaches.

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    Ritchie, Diane L; Barria, Marcelo A; Peden, Alexander H; Yull, Helen M; Kirkpatrick, James; Adlard, Peter; Ironside, James W; Head, Mark W

    2017-04-01

    Creutzfeldt-Jakob disease (CJD) is the prototypic human prion disease that occurs most commonly in sporadic and genetic forms, but it is also transmissible and can be acquired through medical procedures, resulting in iatrogenic CJD (iCJD). The largest numbers of iCJD cases that have occurred worldwide have resulted from contaminated cadaveric pituitary-derived human growth hormone (hGH) and its use to treat primary and secondary growth hormone deficiency. We report a comprehensive, tissue-based and molecular genetic analysis of the largest series of UK hGH-iCJD cases reported to date, including in vitro kinetic molecular modelling of genotypic factors influencing prion transmission. The results show the interplay of prion strain and host genotype in governing the molecular, pathological and temporal characteristics of the UK hGH-iCJD epidemic and provide insights into the adaptive mechanisms involved when prions cross genotypic barriers. We conclude that all of the available evidence is consistent with the hypothesis that the UK hGH-iCJD epidemic resulted from transmission of the V2 human prion strain, which is associated with the second most common form of sporadic CJD.

  6. EEG Differences in Two Clinically Similar Rapid Dementias: Voltage-Gated Potassium Channel Complex-Associated Autoimmune Encephalitis and Creutzfeldt-Jakob Disease.

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    Freund, Brin; Probasco, John C; Cervenka, Mackenzie C; Sutter, Raoul; Kaplan, Peter W

    2018-05-01

    Distinguishing treatable causes for rapidly progressive dementia from those that are incurable is vital. Creutzfeldt-Jakob disease (CJD) and voltage-gated potassium channel complex-associated autoimmune encephalitis (VGKC AE) are 2 such conditions with disparate outcomes and response to treatment. To determine the differences in electroencephalography between CJD and VGKC AE, we performed a retrospective review of medical records and examined clinical data, neuroimaging, and electroencephalographs performed in patients admitted for evaluation for rapidly progressive dementia diagnosed with CJD and VGKC AE at the Johns Hopkins Hospital and Bayview Medical Center between January 1, 2007 and December 31, 2015. More patients in the VGKC AE group had seizures (12/17) than those with CJD (3/14; P = .008). Serum sodium levels were lower in those with VGKC AE ( P = .001). Cerebrospinal fluid (CSF) white blood cell count was higher in VGKC AE ( P = .008). CSF protein 14-3-3 ( P = .018) was more commonly detected in CJD, and tau levels were higher in those with CJD ( P VGKC AE, and electroencephalography can aid in their diagnoses. Performing serial EEGs better delineates these conditions.

  7. 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.

  8. 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.

  9. Geographic exposure risk of variant Creutzfeldt-Jakob disease in US blood donors: a risk-ranking model to evaluate alternative donor-deferral policies.

    Science.gov (United States)

    Yang, Hong; Huang, Yin; Gregori, Luisa; Asher, David M; Bui, Travis; Forshee, Richard A; Anderson, Steven A

    2017-04-01

    Variant Creutzfeldt-Jakob disease (vCJD) has been transmitted by blood transfusion (TTvCJD). The US Food and Drug Administration (FDA) recommends deferring blood donors who resided in or traveled to 30 European countries where they may have been exposed to bovine spongiform encephalopathy (BSE) through beef consumption. Those recommendations warrant re-evaluation, because new cases of BSE and vCJD have markedly abated. The FDA developed a risk-ranking model to calculate the geographic vCJD risk using country-specific case rates and person-years of exposure of US blood donors. We used the reported country vCJD case rates, when available, or imputed vCJD case rates from reported BSE and UK beef exports during the risk period. We estimated the risk reduction and donor loss should the deferral be restricted to a few high-risk countries. We also estimated additional risk reduction by leukocyte reduction (LR) of red blood cells (RBCs). The United Kingdom, Ireland, and France had the greatest vCJD risk, contributing approximately 95% of the total risk. The model estimated that deferring US donors who spent extended periods of time in these three countries, combined with currently voluntary LR (95% of RBC units), would reduce the vCJD risk by 89.3%, a reduction similar to that achieved under the current policy (89.8%). Limiting deferrals to exposure in these three countries would potentially allow donations from an additional 100,000 donors who are currently deferred. Our analysis suggests that a deferral option focusing on the three highest risk countries would achieve a level of blood safety similar to that achieved by the current policy. © 2016 AABB.

  10. PrP mRNA and protein expression in brain and PrP(c) in CSF in Creutzfeldt-Jakob disease MM1 and VV2.

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    Llorens, Franc; Ansoleaga, Belén; Garcia-Esparcia, Paula; Zafar, Saima; Grau-Rivera, Oriol; López-González, Irene; Blanco, Rosi; Carmona, Margarita; Yagüe, Jordi; Nos, Carlos; Del Río, José Antonio; Gelpí, Ellen; Zerr, Inga; Ferrer, Isidre

    2013-01-01

    Creutzfeldt-Jakob disease (CJD) is a heterogenic neurodegenerative disorder associated with abnormal post-translational processing of cellular prion protein (PrP(c)). CJD displays distinctive clinical and pathological features which correlate with the genotype at the codon 129 (methionine or valine: M or V respectively) in the prion protein gene and with size of the protease-resistant core of the abnormal prion protein PrP(sc) (type 1: 20/21 kDa and type 2: 19 kDa). MM1 and VV2 are the most common sporadic CJD (sCJD) subtypes. PrP mRNA expression levels in the frontal cortex and cerebellum are reduced in sCJD in a form subtype-dependent. Total PrP protein levels and PrP(sc) levels in the frontal cortex and cerebellum accumulate differentially in sCJD MM1 and sCJD VV2 with no relation between PrP(sc) deposition and spongiform degeneration and neuron loss, but with microgliosis, and IL6 and TNF-α response. In the CSF, reduced PrP(c), the only form present in this compartment, occurs in sCJD MM1 and VV2. PrP mRNA expression is also reduced in the frontal cortex in advanced stages of Alzheimer disease, Lewy body disease, progressive supranuclear palsy, and frontotemporal lobe degeneration, but PrP(c) levels in brain varies from one disease to another. Reduced PrP(c) levels in CSF correlate with PrP mRNA expression in brain, which in turn reflects severity of degeneration in sCJD.

  11. Comparison of the clinical course of Japanese MM1-type sporadic Creutzfeldt-Jakob disease between subacute spongiform encephalopathy and panencephalopathic-type.

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    Iwasaki, Yasushi; Tatsumi, Shinsui; Mimuro, Maya; Kitamoto, Tetsuyuki; Yoshida, Mari

    2014-06-01

    Approximately half of Japanese sporadic Creutzfeldt-Jakob disease (sCJD) cases show panencephalopathic-type (PE-type) pathology, which is a rare subtype in North Americans and Europeans. Until now, the differences in the clinical course between subacute spongiform encephalopathy (SSE) cases and PE-type cases have been unclear. To investigate the clinical course of both subtypes, clinical findings from 42 Japanese MM1-type sCJD cases (20 SSE cases and 22 PE-type cases) were retrospectively evaluated by statistical analysis. No significant differences could be found regarding age at disease onset, the period between disease onset and first observation of myoclonus, the period between disease onset and the first observation of periodic sharp-wave complexes on electroencephalogram, or the period between disease onset and progression to the akinetic mutism state - whereas total disease duration and the period between the akinetic mutism state and death were significantly longer in PE-type cases. The prolonged disease duration was induced by the extended survival period in the akinetic mutism state. There was a statistically significant difference between the two series regarding performance of tube-feeding, but no statistically significant difference regarding performance of tracheotomy or gastrostomy. None of the cases received mechanical ventilation. We speculate that the most crucial factor of the prolonged survival period of Japanese sCJD cases, particularly in the PE-type, is that the introduction of tube-feeding in the akinetic mutism state leads to the stabilization of the patient's general condition. Copyright © 2014 Elsevier B.V. All rights reserved.

  12. 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 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.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.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.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.

  13. Relation between clinical findings and progression of cerebral cortical pathology in MM1-type sporadic Creutzfeldt-Jakob disease: proposed staging of cerebral cortical pathology.

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    Iwasaki, Yasushi; Tatsumi, Shinsui; Mimuro, Maya; Kitamoto, Tetsuyuki; Hashizume, Yoshio; Yoshida, Mari

    2014-06-15

    In our pathologic observation of the cerebral cortex including the neocortex, hippocampus, and limbic cortex in 43 Japanese patients with MM1-type sporadic Creutzfeldt-Jakob disease, the earliest pathologic finding was spongiform change and next was gliosis. Subsequently, neuropil rarefaction appeared, followed by neuron loss. On the basis of these observations, we propose the following cortical pathologic staging: Stage I, spongiform change; Stage II, hypertrophic astrocytosis; Stage III, neuropil rarefaction; Stage IV, neuron loss; Stage V, status spongiosus; and Stage VI, large cavity formation. We also suggest a more simple staging classification: Stages I and II, mild; Stages III and IV, moderate; and Stages V and VI, severe involvement. Based on statistical analysis of the cases, strong correlation coefficients were obtained between the neocortical and limbic pathologic stage and both total disease duration and brain weight. We estimated that the first observation times of cortical hyperintensity on diffusion-weighted images of magnetic resonance imaging, myoclonus, and periodic sharp wave complexes on the electroencephalogram approximately correspond to the early phase of Stage II of the neocortex. The time to reach the akinetic mutism state approximately corresponds to the middle phase of Stage II of the neocortex. Therefore, we think that approximate clinical manifestations at death, total disease duration, and brain weight can be estimated according to the pathologic stage of the neocortex or limbic cortex. Panencephalopathic-type pathology appeared approximately 12 months after disease onset, and this time approximately corresponds to the middle phase of Stage III of the neocortex. Copyright © 2014 Elsevier B.V. All rights reserved.

  14. The reporting of theoretical health risks by the media: Canadian newspaper reporting of potential blood transmission of Creutzfeldt-Jakob disease

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    Ahmad Nadya

    2004-01-01

    Full Text Available Abstract Background The media play an important role at the interface of science and policy by communicating scientific information to the public and policy makers. In issues of theoretical risk, in which there is scientific uncertainty, the media's role as disseminators of information is particularly important due to the potential to influence public perception of the severity of the risk. In this article we describe how the Canadian print media reported the theoretical risk of blood transmission of Creutzfeldt-Jakob disease (CJD. Methods We searched 3 newspaper databases for articles published by 6 major Canadian daily newspapers between January 1990 and December 1999. We identified all articles relating to blood transmission of CJD. In duplicate we extracted information from the articles and entered the information into a qualitative software program. We compared the observations obtained from this content analysis with information obtained from a previous policy analysis examining the Canadian blood system's decision-making concerning the potential transfusion transmission of CJD. Results Our search identified 245 relevant articles. We observed that newspapers in one instance accelerated a policy decision, which had important resource and health implication, by communicating information on risk to the public. We also observed that newspapers primarily relied upon expert opinion (47 articles as opposed to published medical evidence (28 articles when communicating risk information. Journalists we interviewed described the challenges of balancing their responsibility to raise awareness of potential health threats with not unnecessarily arousing fear amongst the public. Conclusions Based on our findings we recommend that journalists report information from both expert opinion sources and from published studies when communicating information on risk. We also recommend researchers work more closely with journalists to assist them in identifying

  15. An autopsied case of MV2K + C-type sporadic Creutzfeldt-Jakob disease presenting with widespread cerebral cortical involvement and Kuru plaques.

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    Iwasaki, Yasushi; Saito, Yufuko; Aiba, Ikuko; Kobayashi, Atsushi; Mimuro, Maya; Kitamoto, Tetsuyuki; Yoshida, Mari

    2017-06-01

    MV2-type sporadic Creutzfeldt-Jakob disease (sCJD), which was previously called "Kuru-plaque variant", was gradually revealed to have a wide spectrum and has been classified into three pathological subtypes: MV2K, MV2C and MV2K + C. We herein describe the detailed clinical findings and neuropathologic observations from an autopsied MV2K + C-type Japanese sCJD case with widespread cerebral cortical pathology and Kuru plaques. In the early stages of the disease, the patient exhibited gait disturbance with ataxia and dysarthria as well as gradual appearance of cognitive dysfunction. Diffusion-weighted images (DWI) on MRI revealed extensive cerebral cortical hyperintensity. Pathologic investigation revealed extensive spongiform change in the cerebral cortex, particularly in the deeper layers. Vacuole size varied, and some were confluent. Prion protein (PrP) immunostaining revealed extensive PrP deposition in the cerebral cortex, basal ganglia, thalamus, cerebellum, brainstem and spinal cord. In the cerebral cortex, synaptic-type, Kuru plaque-like, and coarse plaque-type PrP depositions were mainly observed, along with some perivacuolar-type PrP depositions. Kuru plaques and coarse plaque-type PrP depositions also were observed in the cerebellar cortex. PrP gene analysis revealed no mutations, and polymorphic codon 129 exhibited Met/Val heterozygosity. Western blot analysis revealed a mixture of intermediate-type PrP Sc and type 2 PrP Sc . Based on previous reports regarding MV2-type sCJD and the clinicopathologic findings of the present case, we speculated that it may be possible to clinically distinguish each MV2 subtype. Clinical presentation of the MV2K + C subtype includes predominant cerebral cortical involvement signs with ataxia and DWI hyperintensity of the cerebral cortex on MRI. © 2016 Japanese Society of Neuropathology.

  16. SCREENING FOR GENETIC CHANGES AND CODON 129 POLYMORPHISM IN PRNP GENE IN HEALTHY SLOVENIAN POPULATION AND SPORADIC CASES OF CREUTZFELDT-JAKOB DISEASE

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    Sava Smerkolj

    2004-11-01

    Full Text Available Background. Prion protein has an important role in development of prion diseases, fatal neurodegenerative disorders. As the codon 129 genotype of the prion protein gene (PRNP is a known susceptibility factor for the diseases, we wanted to determine its distribution in healthy Slovenian population and also in cases of sporadic Creutzfeldt-Jakob disease (sCJD. Furthermore, we wanted to screen the whole gene in order to establish the presence of genetic changes.Methods. We screened 350 DNA samples of healthy blood donors and 12 DNA samples of patients deceased of sCJD. After the amplification and conformation analysis had been done, the gene was sequenced using an automatic sequencer.Results. Methionine homozygotes comprised 46.8% of healthy population, valine homozygotes 12.1% and heterozygotes 41.1%; out of 12 sCJD patients 10 were methionine homozygotes (83.3%, 1 was valine homozygote (8.3% and 1 was heterozygote (8.3%.Found SNPs were combination of codon 76 change (228C > T and codon 84 change (252T > C in a single sample of healthy population, combination of codon 68 change (204T > C and codon 76 change (228C > T in two samples of healthy population and codon 117 change (351A > G in a healthy population sample and in a valine homozygote patient.Conclusions. In comparison to the pooled Caucasian population is genotype M/M frequency slightly increased on account of decreased genotype M/V frequency in healthy Slovenian population, suggesting a little higher risk for acquiring a new variant of CJD (vCJD, because up to date all confirmed vCJD cases except one heterozygote were methionine homozygotes. Codon 129 genotype distribution in sCJD can be described as disease-specific. The absence of pathogenic mutations in sCJD patients confirms the non-familial, sporadic disease form.

  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.

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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.

  18. 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.

  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. © 2013 International Society of Neuropathology.

  2. Atypical Creutzfeldt-Jakob disease with PrP-amyloid plaques in white matter: molecular characterization and transmission to bank voles show the M1 strain signature.

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    Rossi, Marcello; Saverioni, Daniela; Di Bari, Michele; Baiardi, Simone; Lemstra, Afina Willemina; Pirisinu, Laura; Capellari, Sabina; Rozemuller, Annemieke; Nonno, Romolo; Parchi, Piero

    2017-11-23

    Amyloid plaques formed by abnormal prion protein (PrP Sc ) aggregates occur with low frequency in Creutzfeldt-Jakob disease, but represent a pathological hallmark of three relatively rare disease histotypes, namely variant CJD, sporadic CJDMV2K (methionine/valine at PRNP codon 129, PrP Sc type 2 and kuru-type amyloid plaques) and iatrogenic CJDMMiK (MM at codon 129, PrP Sc of intermediate type and kuru plaques). According to recent studies, however, PrP-amyloid plaques involving the subcortical and deep nuclei white matter may also rarely occur in CJDMM1 (MM at codon 129 and PrP Sc type 1), the most common CJD histotype.To further characterize the phenotype of atypical CJDMM1 with white matter plaques (p-CJDMM1) and unravel the basis of amyloid plaque formation in such cases, we compared clinical and histopathological features and PrP Sc physico-chemical properties between 5 p-CJDMM1 and 8 typical CJDMM1 brains lacking plaques. Furthermore, transmission properties after bioassay in two genetic lines of bank voles were also explored in the two groups.All 5 p-CJDMM1 cases had a disease duration longer than one year. Three cases were classified as sporadic CJDMM1, one as sporadic CJDMM1 + 2C and one as genetic CJDE200K-MM1. Molecular mass, protease sensitivity and thermo-solubilization of PrP Sc aggregates did not differ between p-CJDMM1 and classical CJDMM1 cases. Likewise, transmission properties such as incubation time, lesion profile and PrP Sc properties in bank voles also matched in the two groups.The present data further define the clinical-pathologic phenotype of p-CJDMM1, definitely establish it as a distinctive CJD histotype and demonstrate that PrP-plaque formation in this histotype is not a strain-specific feature. Since cases lacking amyloid plaques may also manifest a prolonged (i.e. > than one year) disease course, unidentified, host-specific factors likely play a significant role, in addition to disease duration, in generating white matter Pr

  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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    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

  4. Emergence of two prion subtypes in ovine PrP transgenic mice infected with human MM2-cortical Creutzfeldt-Jakob disease prions.

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    Chapuis, Jérôme; Moudjou, Mohammed; Reine, Fabienne; Herzog, Laetitia; Jaumain, Emilie; Chapuis, Céline; Quadrio, Isabelle; Boulliat, Jacques; Perret-Liaudet, Armand; Dron, Michel; Laude, Hubert; Rezaei, Human; Béringue, Vincent

    2016-02-05

    Mammalian prions are proteinaceous pathogens responsible for a broad range of fatal neurodegenerative diseases in humans and animals. These diseases can occur spontaneously, such as Creutzfeldt-Jakob disease (CJD) in humans, or be acquired or inherited. Prions are primarily formed of macromolecular assemblies of the disease-associated prion protein PrP(Sc), a misfolded isoform of the host-encoded prion protein PrP(C). Within defined host-species, prions can exist as conformational variants or strains. Based on both the M/V polymorphism at codon 129 of PrP and the electrophoretic signature of PrP(Sc) in the brain, sporadic CJD is classified in different subtypes, which may encode different strains. A transmission barrier, the mechanism of which remains unknown, limits prion cross-species propagation. To adapt to the new host, prions have the capacity to 'mutate' conformationally, leading to the emergence of a variant with new biological properties. Here, we transmitted experimentally one rare subtype of human CJD, designated cortical MM2 (129 MM with type 2 PrP(Sc)), to transgenic mice overexpressing either human or the VRQ allele of ovine PrP(C). In marked contrast with the reported absence of transmission to knock-in mice expressing physiological levels of human PrP, this subtype transmitted faithfully to mice overexpressing human PrP, and exhibited unique strain features. Onto the ovine PrP sequence, the cortical MM2 subtype abruptly evolved on second passage, thereby allowing emergence of a pair of strain variants with distinct PrP(Sc) biochemical characteristics and differing tropism for the central and lymphoid tissues. These two strain components exhibited remarkably distinct replicative properties in cell-free amplification assay, allowing the 'physical' cloning of the minor, lymphotropic component, and subsequent isolation in ovine PrP mice and RK13 cells. Here, we provide in-depth assessment of the transmissibility and evolution of one rare subtype of

  5. Use of 14-3-3 and other brain-specific proteins in CSF in the diagnosis of variant Creutzfeldt-Jakob disease

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    Green, A; Thompson, E; Stewart, G; Zeidler, M; McKenzie, J; MacLeod, M; Ironside, J; Will, R; Knight, R

    2001-01-01

    OBJECTIVES—The detection of the protein 14-3-3 in the CSF has been shown to be a reliable and sensitive marker for sporadic Creutzfeldt-Jakob disease (CJD). Other brain-specific proteins such as neuron specific enolase (NSE), S-100b, and tau protein have also been reported to be increased in the CSF of patients with sporadic CJD. In 1996a variant of CJD (vCJD) was described which is likely to be causally linked to the bovine spongiform encephalopathy agent. This study reports and compares the findings of CSF brain specific protein analysis in 45 patients with vCJD and in 34 control patients.
METHODS—The CSF from 45 patients with vCJD and 34 controls were investigated for the presence of 14-3-3 by SDS-polyacrylamide gel electrophoresis (SDS-PAGE) and western blotting with chemiluminescent detection. Tau protein, S-100b, and NSE concentrations in CSF were measured using enzyme immunoassays.
RESULTS—Protein 14-3-3 was detected in the CSF of 22/45 patients with vCJD and in 3/34 controls. The mean concentrations of NSE, S-100b, and tau protein in CSF were significantly raised in patients with vCJD compared with controls. The positive predictive value of CSF 14-3-3 was 86% and the negative predictive value was 63%. These values are lower than those reported for sporadic CJD. An increased CSF tau had a positive predictive value of 93% and a negative predictive value of 81%. The combination of CSF 14-3-3 and/or increased CSF tau had a positive predictive value of 91% and a negative predictive value of 84%.
CONCLUSIONS—CSF protein 14-3-3 is not as useful a marker for vCJD as it is for sporadic CJD. Increased concentration of CSF tau was found to be a sensitive marker of vCJD but as concentrations may be increased in many forms of non-CJD dementia, this may limit its usefulness as a diagnostic test.

 PMID:11385008

  6. 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

  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

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    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. Insights into the management of emerging infections: regulating variant Creutzfeldt-Jakob disease transfusion risk in the UK and the US.

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    Ponte, Maya L

    2006-10-01

    Variant Creutzfeldt-Jakob disease (vCJD) is a human prion disease caused by infection with the agent of bovine spongiform encephalopathy. After the recognition of vCJD in the UK in 1996, many nations implemented policies intended to reduce the hypothetical risk of transfusion transmission of vCJD. This was despite the fact that no cases of transfusion transmission had yet been identified. In December 2003, however, the first case of vCJD in a recipient of blood from a vCJD-infected donor was announced. The aim of this study is to ascertain and compare the factors that influenced the motivation for and the design of regulations to prevent transfusion transmission of vCJD in the UK and US prior to the recognition of this case. A document search was conducted to identify US and UK governmental policy statements and guidance, transcripts (or minutes when transcripts were not available) of scientific advisory committee meetings, research articles, and editorials published in medical and scientific journals on the topic of vCJD and blood transfusion transmission between March 1996 and December 2003. In addition, 40 interviews were conducted with individuals familiar with the decision-making process and/or the science involved. All documents and transcripts were coded and analyzed according to the methods and principles of grounded theory. Data showed that while resulting policies were based on the available science, social and historical factors played a major role in the motivation for and the design of regulations to protect against transfusion transmission of vCJD. First, recent experience with and collective guilt resulting from the transfusion-transmitted epidemics of HIV/AIDS in both countries served as a major, historically specific impetus for such policies. This history was brought to bear both by hemophilia activists and those charged with regulating blood products in the US and UK. Second, local specificities, such as the recall of blood products for possible

  9. Insights into the management of emerging infections: regulating variant Creutzfeldt-Jakob disease transfusion risk in the UK and the US.

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    Maya L Ponte

    2006-10-01

    Full Text Available Variant Creutzfeldt-Jakob disease (vCJD is a human prion disease caused by infection with the agent of bovine spongiform encephalopathy. After the recognition of vCJD in the UK in 1996, many nations implemented policies intended to reduce the hypothetical risk of transfusion transmission of vCJD. This was despite the fact that no cases of transfusion transmission had yet been identified. In December 2003, however, the first case of vCJD in a recipient of blood from a vCJD-infected donor was announced. The aim of this study is to ascertain and compare the factors that influenced the motivation for and the design of regulations to prevent transfusion transmission of vCJD in the UK and US prior to the recognition of this case.A document search was conducted to identify US and UK governmental policy statements and guidance, transcripts (or minutes when transcripts were not available of scientific advisory committee meetings, research articles, and editorials published in medical and scientific journals on the topic of vCJD and blood transfusion transmission between March 1996 and December 2003. In addition, 40 interviews were conducted with individuals familiar with the decision-making process and/or the science involved. All documents and transcripts were coded and analyzed according to the methods and principles of grounded theory. Data showed that while resulting policies were based on the available science, social and historical factors played a major role in the motivation for and the design of regulations to protect against transfusion transmission of vCJD. First, recent experience with and collective guilt resulting from the transfusion-transmitted epidemics of HIV/AIDS in both countries served as a major, historically specific impetus for such policies. This history was brought to bear both by hemophilia activists and those charged with regulating blood products in the US and UK. Second, local specificities, such as the recall of blood

  10. Creutzfeldt-Jakob disease (image)

    Science.gov (United States)

    ... called a prion. Loss of brain function resembles Alzheimer's disease, but is very rapid in progression. Complete dementia usually occurs by the sixth month, death follows quickly. There is no known cure.

  11. Preserved regional cerebral blood flow in the occipital cortices, brainstem, and cerebellum of patients with V180I-129M genetic Creutzfeldt-Jakob disease in serial SPECT studies.

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    Hayashi, Yuichi; Yoshikura, Nobuaki; Takekoshi, Akira; Yamada, Megumi; Asano, Takahiko; Kimura, Akio; Satoh, Katsuya; Kitamoto, Tetsuyuki; Inuzuka, Takashi

    2016-11-15

    Creutzfeldt-Jakob disease (CJD) with a causative point mutation of valine to isoleucine at codon 180 (V180I) is one of the major types of genetic CJD (gCJD) in Japan. V180I gCJD is rarely accompanied by a family history, and its clinical characteristics include late-onset, long disease duration, and edematous cortical hyperintensity in diffusion, fluid attenuate inversion and T2-weighted MRI. We performed serial imaging with single-photon emission computed tomography (SPECT) and MRI in three V180I gCJD cases over long-term observation. All cases were characterized by progressive dementia, parkinsonism, and the absence of cerebellar signs or cortical visual dysfunction in their clinical courses. Moreover, during the end-stage, SPECT findings showed preserved regional cerebral blood flow (rCBF) in the occipital cortices, brainstem, and cerebellum. Similarly, no apparent atrophy or increased signal intensities were observed in MRI images of the occipital and cerebellar regions. In conclusion, we report a decrease in rCBF predominantly in the frontal and temporal cortices during the early-stage, which became more widespread as the disease progressed. Importantly, rCBF was preserved in the occipital cortices, brainstem, and cerebellar regions until the end-stage, which may be distinct to V180I gCJD cases. Copyright © 2016 Elsevier B.V. All rights reserved.

  12. 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.

  13. 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?

  14. Absence of Evidence for a Causal Link between Bovine Spongiform Encephalopathy Strain Variant L-BSE and Known Forms of Sporadic Creutzfeldt-Jakob Disease in Human PrP Transgenic Mice.

    Science.gov (United States)

    Jaumain, Emilie; Quadrio, Isabelle; Herzog, Laetitia; Reine, Fabienne; Rezaei, Human; Andréoletti, Olivier; Laude, Hubert; Perret-Liaudet, Armand; Haïk, Stéphane; Béringue, Vincent

    2016-12-01

    Prions are proteinaceous pathogens responsible for subacute spongiform encephalopathies in animals and humans. The prions responsible for bovine spongiform encephalopathy (BSE) are zoonotic agents, causing variant Creutzfeldt-Jakob disease (CJD) in humans. The transfer of prions between species is limited by a species barrier, which is thought to reflect structural incompatibilities between the host cellular prion protein (PrP C ) and the infecting pathological PrP assemblies (PrP Sc ) constituting the prion. A BSE strain variant, designated L-BSE and responsible for atypical, supposedly spontaneous forms of prion diseases in aged cattle, demonstrates zoonotic potential, as evidenced by its capacity to propagate more easily than classical BSE in transgenic mice expressing human PrP C and in nonhuman primates. In humanized mice, L-BSE propagates without any apparent species barrier and shares similar biochemical PrP Sc signatures with the CJD subtype designated MM2-cortical, thus opening the possibility that certain CJD cases classified as sporadic may actually originate from L-type BSE cross-transmission. To address this issue, we compared the biological properties of L-BSE and those of a panel of CJD subtypes representative of the human prion strain diversity using standard strain-typing criteria in human PrP transgenic mice. We found no evidence that L-BSE causes a known form of sporadic CJD. Since the quasi-extinction of classical BSE, atypical BSE forms are the sole BSE variants circulating in cattle worldwide. They are observed in rare cases of old cattle, making them difficult to detect. Extrapolation of our results suggests that L-BSE may propagate in humans as an unrecognized form of CJD, and we urge both the continued utilization of precautionary measures to eliminate these agents from the human food chain and active surveillance for CJD phenotypes in the general population. Copyright © 2016, American Society for Microbiology. All Rights Reserved.

  15. 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.

  16. Diagnostic Accuracy of a Combined Analysis of Cerebrospinal Fluid t-PrP, t-tau, p-tau, and Aβ42 in the Differential Diagnosis of Creutzfeldt-Jakob Disease from Alzheimer's Disease with Emphasis on Atypical Disease Variants.

    Science.gov (United States)

    Abu Rumeileh, Samir; Lattanzio, Francesca; Stanzani Maserati, Michelangelo; Rizzi, Romana; Capellari, Sabina; Parchi, Piero

    2017-01-01

    According to recent studies, the determination of cerebrospinal fluid (CSF) total tau (t-tau)/phosphorylated tau (p-tau) ratio and total prion protein (t-PrP) levels significantly improves the accuracy of the diagnosis of Alzheimer's disease (AD) in atypical cases with clinical or laboratory features mimicking Creutzfeldt-Jakob disease (CJD). However, this has neither been validated nor tested in series including atypical CJD variants. Furthermore, the added diagnostic value of amyloid-β (Aβ)42 remains unclear. To address these issues, we measured t-PrP, 14-3-3, t-tau, p-tau, and Aβ42 CSF levels in 45 typical and 44 atypical/rapidly progressive AD patients, 54 typical and 54 atypical CJD patients, and 33 controls. CJD patients showed significantly lower CSF t-PrP levels than controls and AD patients. Furthermore, atypical CJD was associated with lower t-PrP levels in comparison to typical CJD. T-tau, 14-3-3, or t-PrP alone yielded, respectively, 80.6, 63.0, and 73.0% sensitivity and 75.3, 92.1, and 75% specificity in distinguishing AD from CJD. On receiver operating characteristic (ROC) curve analyses of biomarker combinations, the (t-tau×Aβ42)/(p-tau×t-PrP) ratio achieved the best accuracy, with 98.1% sensitivity and 97.7% specificity overall, and 96.2% sensitivity and 95.5% specificity for the "atypical" disease groups. Our results show that the combined analysis of CSF t-PrP, t-tau, p-tau, and Aβ42 is clinically useful in the differential diagnosis between CJD and AD. Furthermore, the finding of reduced CSF t-PrP levels in CJD patients suggest that, likewise Aβ42 in AD, CSF t-PrP levels reflect the extent of PrPc conversion into abnormal PrP (PrPSc) and the burden of PrPSc deposition in CJD.

  17. Bruk av urtemedisiner blant gravide i Mali : Intervju av 72 healere i Bamako, Siby og Dioila

    OpenAIRE

    Al-Zayadi, Waled

    2012-01-01

    WHO’s rapport fra 2001 viser til at hele 75 % av Mali sin populasjon bruker tradisjonell medisin. Det er beregnet til å være 1 medisinmann pr 500 innbyggere. Hovedmålet med denne oppgaven var å undersøke tradisjonelle medisinmenns behandling av sykdom og plager i svangerskapet i Mali. Det er lite som vites om bruk av urtemedisiner blant gravide i Mali. Det er heller ingen studier gjort fra healerens side. Studien var med på å kartlegge hvilke urter som brukes til den enkelte plagen. I ette...

  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. Copyright © 2015 Elsevier B.V. All rights reserved.

  19. Spa-typing av stammer av Staphylococcus aureus fra blodkulturer analysert ved UNN Tromsø 2007-2008

    OpenAIRE

    Midtgard, Anette

    2011-01-01

    Bakgrunn: Staphylococcus aureus er et av de vanligste patogenene som kan skape sykdom hos mennesket. Mange av oss er bærere av bakterien i nese eller hals, og det er vist at persisterende bærere har høyere risiko for å utvikle S. aureus-infeksjoner enn andre. De fleste S. aureus-infeksjonene blant bærere er forårsaket av pasientens egen bakteriestamme. Materiale og metode: 140 blodkulturisolater fra pasienter med S. aureus bakteriemi analysert ved Avdeling for mikrobiologi og smitte...

  20. Pårørendes erfaringer med bruk av frivillige ved alvorlig sykdom i hjemmet

    Directory of Open Access Journals (Sweden)

    Sylvi Monika Flateland

    2017-03-01

    Full Text Available Next-of-kins’ experiences when using volunteers for palliative patients living at home The aim of this study was to illuminate next-of-kins’ experiences when using volunteers supplementing community health care services for palliative patients living at home. The study has a qualitative approach. Nine individual interviews were conducted with next of kin from a municipality in Southern Norway. Use of volunteers for palliative care in the home was a relief for next-of-kin, especially prominent for families who had limited social network. Planned volunteering resulted in the best results. Use of volunteer was also found helpful for families as they were given a "breathing space" and a certain normalization of everyday life. The volunteers followed up activities related to the patient that the next-of-kin to a limited extent managed to attend. This affected the next-of-kin’s conscience in a positive way. The next-of-kin experienced the volunteers to be empathetic, they could inspire confidence and showed good characteristics to assist ill people and their next-of-kin. The next-of-kin needed regular relief from volunteers as a supplement to the community health care services. The volunteers were well suited to meet people, who were in a difficult situation, and with their approach they had strong confidence in the next-of-kin and gave them relief in a challenging environment. The coordinator for palliative care was a key person in relation to the link between families, patients in palliative care and the volunteers.

  1. 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

  2. Effekt av ulike kjemiske- og biologiske preparater på forekomsten av Fusarium og mykotoksiner i havre

    OpenAIRE

    Shakery, Bita

    2013-01-01

    Aksfusariose er en vanlig sykdom på korn forårsaket av flere ulike Fusarium-arter. En Fusarium-infeksjon på akset gir tap av avling i tillegg til en reduksjon i kvaliteten på kornet. Reduksjonen i kvalitet skjer i hovedsak gjennom produksjon av en rekke toksiske metabolitter (mykotoksiner) som kan være en helserisiko for både dyr og mennesker. I de senere årene har det vært økt fokus på Fusarium og mykotoksiner i korn både i Norge og i store deler av verden. En årsak til det...

  3. 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.

  4. 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

  5. Creutzfeldt-Jakob disease. What is the role of magnetic resonance tomography?; Creutzfeldt-Jakob-Krankheit. Welche Rolle spielt die MR-Tomographie?

    Energy Technology Data Exchange (ETDEWEB)

    Kujat, C. [Inst. fuer Neuroradiologie der Univ. des Saarlandes, Homburg (Germany); Hagen, T. [Inst. fuer Neuroradiologie der Univ. des Saarlandes, Homburg (Germany); Feiden, W. [Abt. fuer Neuropathologie der Univ. des Saarlandes, Homburg (Germany)

    1995-11-01

    We report three patients with histologically confirmed CJD and confirm that MRI is a valuable tool for the diagnosis of this disease. (orig./MG) [Deutsch] Anhand der MRT-Ergebnisse von 3 Patienten mit histologisch gesicherter CJD und einer Literaturrecherche wird untersucht, ob die MRT einen Beitrag zur Diagnostik der CJD leisten kann. (orig./MG)

  6. 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...

  7. 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.

  8. Etiologic and diagnostic facets of Creutzfeldt-Jakob disease: The effect of genes and environment

    NARCIS (Netherlands)

    P.J. Sánchez-Juan

    2007-01-01

    textabstractTransmissible spongiform encephalopathies (TSE) or prion diseases constitute a fascinating group of neurological disorders. In spite of their rarity, their unique etiopathological mechanisms and epidemiological aspects have attracted a considerable number of researchers. This

  9. MM1+2C sporadic Creutzfeldt-Jakob disease presenting as rapidly progressive nonfluent aphasia.

    Science.gov (United States)

    Allegri, Ricardo F; Bartoloni, Leonardo; Iturry, Mónica; Romero, Carlos; Begué, Christián; Sevlever, Gustavo; Taratuto, Ana Lía

    2014-01-01

    We report a 77-year-old man, presenting with progressive aphasia as an initial symptom, who developed severe dementia over the course of 20 months. Frontal cortex PrPSc western blot was type 2 and codon 129 was MM; brain neuropathology showed cortical vacuoles with perivacuolar PrP immunostaining characteristic of MM2C. Cerebellum showed focal coarse, patchy staining in different sections of the molecular layer, diffuse fine punctuate and coarse PrP immunopositive deposits in the granule cell layer, and focal synaptic immunostaining in the molecular layer, suggestive of MM1+2C by histotyping. This clinical presentation has not yet been described in an MM1+2C subtype by histotyping.

  10. 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...... correlate with regional pathology. Overloading of this system might impair the function of lysosomal enzymes and thus may mimic some features of lysosomal storage disorders. Udgivelsesdato: 2007-Jul...

  11. Wernicke-Korsakoff syndrome as a rare phenotype of sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Bielewicz, Joanna; Szczepańska-Szerej, Anna; Ogórek, Magdalena; Dropko, Piotr; Wojtal, Katarzyna; Rejdak, Konrad

    2018-03-04

    We reported the case of a patient with Wernicke-Korsakoff syndrome (WKs) as an early clinical manifestation of sporadic Creutzfeld-Jakob disease (sCJD). The 66-year-old female complained of dizziness and imbalance which mostly occurred while walking. A neurological examination revealed a triad of symptoms characteristic for WKs such as gaze paresis, ataxia of limbs and trunk as well as memory disturbances with confabulations. The disturbances increased during the course of the disease, which led to the death of the patient four months after the appearance of the signs. The patient was finally diagnosed with sCJD disease. The most useful ancillary examination results supporting sCJD diagnosis were brain diffusion DWI MRI (diffusion weighted magnetic resonance imaging) and the presence of 14-3-3 protein in CSF (cerebrospinal fluid). Since that manifestation of sCJD is very unique other causes should be taken into consideration while making a final diagnosis.

  12. Evaluering av medisinsk behandling: Hva vet vi, og hvordan bør kunnskapen håndteres?

    Directory of Open Access Journals (Sweden)

    Jan Magnus Bjordal

    2009-09-01

    Full Text Available Kunnskap om effekten av medisinsk behandling fremskaffes i kliniske studier. Godt planlagte og gjennomførte randomiserte, placebokontrollerte studier tillegges størst vekt når data skal vurderes. De datamengdene vi besitter når det gjelder forskjellige behandlinger kan oppsummeres i oversikter eller terapianbefalinger som ofte blir normative for klinisk praksis. Dette stiller store krav til evalueringsprosedyrene. I denne oversiktsartikkelen diskuteres i innledningen noen eksempler på at kunnskapsgrunnlaget er blitt feiltolket. Deretter omtales kvalitetskriterier som legges til grunn i vurderingen av data, og den metodologiske basis for meta-analyser. Usikkerhetsmomenter og potensielle fallgruver ved bruk av meta-analyser omtales. Meta-analysens fortrinn eksemplifiseres ved bruk av data om behandling av korsryggsmerter, hvor det nylig er utarbeidet kliniske retningslinjer som etter vår oppfatning er beheftet med feil eller mangler. Meta-analysen har vesentlige fortrinn fremfor mindre formaliserte metoder for oppsummering av kunnskap om terapi ved sykdom

  13. [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.

  14. Diagnosing Sporadic Creutzfeldt-Jakob Disease: Accuracy of CSF 14-3-3 Protein Test of the Spinal Fluid

    Science.gov (United States)

    ... at about age 60 years. Early symptoms include: • Memory problems • Behavior/personality changes • Loss of muscle coordination • Vision problems • Insomnia (difficulty falling asleep or staying asleep) • Depression • Unusual sensations As the disease progresses, the person usually develops ...

  15. EEG?EMG polygraphic study of dystonia and myoclonus in a case of Creutzfeldt?Jakob disease ?

    OpenAIRE

    Hashimoto, Takao; Iwahashi, Teruaki; Ishii, Wataru; Yamamoto, Kanji; Ikeda, Shu-ichi

    2015-01-01

    We report on a patient with sporadic Creutzfeldt–Jakob disease (CJD) who showed dystonia, periodic myoclonus, and periodic sharp wave complexes (PSWCs) on EEG. The EEG–EMG polygraphic study revealed that dystonia appeared without relation to periodic myoclonus and PSWCs and that dystonia EMGs were strongly suppressed after periodic myoclonus EMGs. These findings suggest that dystonia has a pathogenesis different from that of periodic myoclonus and PSWCs, but dystonia and periodic myoclonus ma...

  16. 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

    those in other countries, has a multidisciplinary approach, which is labour-intensive and time-consuming but ensures the most complete set of information possible. With this approach we think that patients with variant CJD would have been detected had they occurred in Denmark. Certain aspects of CJD...

  17. 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.

  18. CT and MRI findings of Creutzfeldt-Jakob disease in the early stage. The usefulness of diffusion-weighted images

    International Nuclear Information System (INIS)

    Ukisu, Ryutaro; Kushihashi, Tamio; Gokan, Takehiko

    2001-01-01

    To detect subtle CT and MRI features of Creutzfeldt-Jacob disease (CJD) in the early stage is important to prevent a human-to-human transmission. This study included 10 patients of CJD who underwent CT and/or MRI in its early stage. CT, T1- and T2-weighted MRI, DWI, and FLAIR images were obtained in 10, 6, 4, and 2 patients respectively. On DWI, abnormal hyperintensities were observed in both cerebral cortex, and in basal ganglia in all patients. On FLAIR images, abnormal hyperintensies were observed in one patient. Detection of abnormal intensities may be possible in the early stage of CJD using MRI, particularly with DWI. (author)

  19. 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.

  20. CT and MRI findings of Creutzfeldt-Jakob disease in the early stage. The usefulness of diffusion-weighted images

    Energy Technology Data Exchange (ETDEWEB)

    Ukisu, Ryutaro; Kushihashi, Tamio; Gokan, Takehiko [Showa Univ., Tokyo (Japan). School of Medicine] [and others

    2001-02-01

    To detect subtle CT and MRI features of Creutzfeldt-Jacob disease (CJD) in the early stage is important to prevent a human-to-human transmission. This study included 10 patients of CJD who underwent CT and/or MRI in its early stage. CT, T1- and T2-weighted MRI, DWI, and FLAIR images were obtained in 10, 6, 4, and 2 patients respectively. On DWI, abnormal hyperintensities were observed in both cerebral cortex, and in basal ganglia in all patients. On FLAIR images, abnormal hyperintensies were observed in one patient. Detection of abnormal intensities may be possible in the early stage of CJD using MRI, particularly with DWI. (author)

  1. AUTOMATION AV GALLERKLIPPARE

    OpenAIRE

    Taboada Vargas, Marco Antonio

    2016-01-01

    Detta examensarbete handlar om automatisering och assistans att CE-märka en maskin. Arbetet innefattade automationsplanering, tillverkning av elskåp, montering på maskin och igångkörning. Automationsplaneringen bestod av elritningar och programmering. Elsystemet bestod av elskåp med bl.a. programmerbar logik, touch-panel och säkerhetskomponenter. CE-märkning utfördes enligt de direktiv, standarder och säkerhetsföreskrifter som bör följas. Nu med det nya automatiserade systemet är samm...

  2. 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...

  3. Forenkling av tekniske systemer

    DEFF Research Database (Denmark)

    Førland-Larsen, Arne; Bramslev, Katharina; Halderaker, Ingrid

    De fleste moderne kontorbygg har omfattende tekniske installasjoner. Mange byggeiere opplever at dagens kompliserte tekniske anlegg ikke fungerer som de skal. De ender med å få reklamasjoner, høyt energiforbruk og klager på inneklima. Kan en kraftig forenkling av ventilasjons-, oppvarmings- og...

  4. 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. Hvor...

  5. 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.

  6. Validering av Evolution 220

    OpenAIRE

    Krakeli, Tor-Arne

    2013-01-01

    - Det har blitt kjøpt inn et nytt spektrofotometer (Evolution 220, Thermo Scientific) til BioLab Nofima. I den forbindelsen har det blitt utført en validering som involverer kalibreringsstandarder fra produsenten og en test på normal distribusjon (t-test) på to metoder (Total fosfor, Tryptofan). Denne valideringen fant Evolution 220 til å være et akseptabelt alternativ til det allerede benyttede spektrofotometeret (Helios Beta). På bakgrunn av noen instrumentbegrensninger må de aktuelle an...

  7. 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

  8. 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

  9. 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...

  10. Mödrars upplevelser av att drabbas av postpartumdepression

    OpenAIRE

    Wallin, Rebecca; Pitkämäki, Kia

    2016-01-01

    Bakgrund: Postpartumdepression (PPD) drabbar mellan 8-15% av alla kvinnor som föder barn och innebär stora påfrestningar för både modern, partnern och spädbarnet. Utvecklandet av PPD har multifaktoriella orsaker som tidigare psykiatrisk sjukdom, dåligt parförhållande och brist på socialt stöd. Syfte: Studiens syfte var att undersöka kvinnors upplevelser av postpartumdepression. Metod: Studien är en litteraturöversikt där vetenskapliga artiklar motsvarande studiens syfte granskades. Samtliga a...

  11. Verdsettelse av Bremnes Seashore AS

    OpenAIRE

    Selle, Simon Flatebø

    2017-01-01

    Siden etablering i 1946 har Bremnes Seashore AS ønsket å levere verdens beste lakseprodukt. Det startet i det små med pigghå og regnbueørret før det i 1970 ble satset på det vi i dag kjenner som kommersiell lakseoppdrett. Bremnes beskrives av Innovasjon Norge som bransjerevolusjonær. Spesielt viktig var deres utvikling og implementering av pre-rigor foredling som i dag utgjør standarden for ethvert moderne fiskeslakteri. I nyere tid ønsker de å gjøre det igjen med utvikling av ...

  12. 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....

  13. Kuru

    Science.gov (United States)

    ... type to also change shape. Other TSEs include Creutzfeldt-Jakob disease and fatal familial insomnia in humans, bovine spongiform ... type to also change shape. Other TSEs include Creutzfeldt-Jakob disease and fatal familial insomnia in humans, bovine spongiform ...

  14. Motorsystem : Roderstyrning av autonom segelrobot

    OpenAIRE

    Frank, Morgan

    2017-01-01

    Examensarbetet har gjorts på begäran av ÅSR (Åland Sailing Robots) genom Anna Friebe. Syftet med arbetet är att skapa energisnålt motorsystem för styrning av vindflöjeln och roder till 4 meter lång segelrobot som ägs av ÅSR. Ett exemplar av den styranordningen som jag anser kommer att fungera bäst för en robotsegelbåt har konstruerats och kopplats upp på testbänk där jag sedan har lämnat över arbetet för programmering. I detta arbete har jag undersökt motorer, givare, motorstyrni...

  15. Effektivisering av arbetet med rumsbeskrivningar

    OpenAIRE

    Enström, Magnus

    2016-01-01

    För att ta byggbranschen till nästa steg i utvecklingen är BIM det naturliga steget. Iteorin tycks många av lösningarna som medföljer implementationen av BIM varaenkla att förstå och skapa, i praktiken ligger dock de tekniska lösningarna långt ifrånen full implementation i branschen. I arbetet med olika typer av beskrivningar inomett byggprojekt har utvecklingen med hjälp av BIM stått still länge. Då det inte finnsnågot vedertaget sätt att utnyttja en BIM-modells information för att fylla oli...

  16. Astrometry VLBI in Space (AVS

    Science.gov (United States)

    Altunin, V.; Alekseev, V.; Akim, E.; Eubanks, M.; Kingham, K.; Treuhaft, R.; Sukhanov, K.

    1995-01-01

    A proposed new space radio astronomy mission for astrometry is described. The Astrometry VLBI (very long baseline) in Space (AVS) nominal mission includes two identical spacecraft, each with a 4-m antenna sending data to a 70-m ground station. The goals of AVS are improving astrometry accuracy to the microarcsecond level and improving the accuracy of the transformation between the inertial radio and optical coordinate reference frames.

  17. Astrometry VLBI in Space (AVS)

    Science.gov (United States)

    Cheng, Li-Jen; Reyes, George

    1995-01-01

    This paper describes a proposal for a new space radio astronomy mission for astrometry using Very Long Baseline Interferometry (VLBI) called Astrometry VLBI in Space (AVS). The ultimate goals of AVS are improving the accuracy of radio astrometry measurements to the microarcsecond level in one epoch of measurements and improving the accuracy of the transformation between the inertial radio and optical coordinate reference frames. This study will also assess the impact of this mission on astrophysics astrometry and geophysics.

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

    OpenAIRE

    Díaz Martínez, Juan Carlos; Yuri, Takeuchi Tan

    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...

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

    Directory of Open Access Journals (Sweden)

    R. Williams

    2011-01-01

    Full Text Available Cognitive decline affects one in twenty people over the age of 65. There is often a paucity of clues as to the underlying pathology, and while the diagnosis will usually prove to be either Alzheimer’s disease or vascular dementia, there may be clinical features suggesting rarer alternatives. This case of a 71-year-old lady with a 3-month history of progressive cognitive decline illustrates clinical features suggestive of Creutzfeltd-Jakob disease such as rapid decline in conscious level and myoclonic jerking. Diagnosis was confirmed by 3 means: (1 Electroencephalogram demonstrating periodic sharp wave complexes, (2 MRI brain showing cortical ribboning and high signal in the caudate nucleus, and (3 presence of protein S100 and protein14-3-3 in the cerebrospinal fluid. Postmortem brain histology confirmed a typical spongiform encephalopathy. Establishing an underlying aetiology is dementia is important not only for prognostic reasons but in order to detect potentially reversible causes. In cases of an atypical dementing illness our proposed investigations may assist in confirming or excluding underlying Creutzfeltd-Jakob disease.

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

    Science.gov (United States)

    Mille, Caroline; Debarnot, Dominique; Zorzi, Willy; Moualij, Benaissa El; Quadrio, Isabelle; Perret-Liaudet, Armand; Coudreuse, Arnaud; Legeay, Gilbert; Poncin-Epaillard, Fabienne

    2012-01-01

    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. PMID:25586034

  1. Observance of Sterilization Protocol Guideline Procedures of Critical Instruments for Preventing Iatrogenic Transmission of Creutzfeldt-Jakob Disease in Dental Practice in France, 2017

    Directory of Open Access Journals (Sweden)

    Denis Bourgeois

    2018-04-01

    Full Text Available Effective sterilization of reusable instruments contaminated by Creutzfeldt–Jakob disease in dental care is a crucial issue for public health. The present cross-sectional study investigated how the recommended procedures for sterilization were implemented by French dental practices in real-world settings. A sample of dental practices was selected in the French Rhône-Alpes region. Data were collected by a self-questionnaire in 2016. Sterilization procedures (n = 33 were classified into 4 groups: (1 Pre-sterilization cleaning of reusable instruments; (2 Biological verification of sterilization cycles—Monitoring steam sterilization procedures; (3 Autoclave performance and practitioner knowledge of autoclave use; (4 Monitoring and documentation of sterilization procedures—Tracking and tracing the instrumentation. Answers were provided per procedure, along with the global implementation of procedures within a group (over 80% correctly performed. Then it was verified how adherence to procedure groups varied with the size of the dental practice and the proportion of dental assistants within the team. Among the 179 questionnaires available for the analyses, adherence to the recommended procedures of sterilization noticeably varied between practices, from 20.7% to 82.6%. The median percentages of procedures correctly implemented per practice were 58.1%, 50.9%, 69.2% and 58.2%, in Groups 1, 2, 3 and 4, respectively (corresponding percentages for performing over 80% of the procedures in the group: 23.4%, 6.6%, 46.6% and 38.6%. Dental practices ≥ 3 dental units performed significantly better (>80% procedures of Groups 2 and 4 (p = 0.01 and p = 0.002, respectively, while no other significant associations emerged. As a rule, practices complied poorly with the recommended procedures, despite partially improved results in bigger practices. Specific training regarding sterilization procedures and a better understanding of the reasons leading to their non-compliance are needed.

  2. Human growth hormone-related latrogenic Creutzfeldt-Jakob disease: Search for a genetic susceptibility by analysis of the PRNP coding region

    Energy Technology Data Exchange (ETDEWEB)

    Jaegly, A.; Boussin, F.; Deslys, J.P. [CEA/CRSSA/DSV/DPTE, Fontenay-aux-Roses (France)] [and others

    1995-05-20

    The human PRNP gene encoding PrP is located on chromosome 20 and consists of two exons and a single intron. The open reading frame is entirely fitted into the second exon. Genetic studies indicate that all of the familial and several sporadic forms of TSSEs are associated with mutations in the PRNP 759-bp coding region. Moreover, homozygosity at codon 129, a locus harboring a polymorphism among the general population, was proposed as a genetic susceptibility marker for both sporadic and iatrogenic CJD. To assess whether additional genetic predisposition markers exist in the PRNP gene, the authors sequenced the PRNP coding region of 17 of the 32 French patients who developed a hGH-related CJD.

  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. Klubbhusverksamheten - Betydelse av dagligt arbete

    OpenAIRE

    Niskanen, Karolina

    2011-01-01

    Syftet med undersökningen är att beskriva klubbhusmedlemmarnas uppfattning om det dagliga arbetet och dess betydelse för välmående i vardagen. Undersökningen utfördes i form av kvalitativa intervjuer med fem medlemmar av klubbhuset Pelaren i Mariehamn. Forskningsfrågorna som skulle besvaras var hur klubbhusmedlemmarna uppfattar arbete, vad arbete har för betydelse för deras hälsa, vilka aktiviteter de upplever som betydelsefulla i klubbhusverksamheten samt vad i klubbhusmiljön som stöder dem ...

  5. Simulering av muskelaktivitet vid pedalbromsning

    OpenAIRE

    Ejdepalm, Erik; Westerdahl, Walter

    2009-01-01

    En muskeloskeletal kroppsmodell har undersökts med avseende på maximal muskelbelastning genom biomekanisk simulering i programmet AnyBody. En kroppsmodell har låtits interagera med ett reglage i form av en bromspedal från en Saab 9-3 och muskelbelastningen till följd av att pedalen trampas ned har minimerats. De parametrar hos pedalen som har varierats är initialvinkeln och returfjäderns fjäderkonstant. Den sits på vilken kroppsmodellen sitter har flyttats vertikalt och horisontellt i förhåll...

  6. 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...

  7. Analyse av LOD-tiltak

    OpenAIRE

    Kunduraci, Meltem

    2016-01-01

    Endrede klimatiske forhold og større urbanisering medfører økte oversvømmelsesskader i urbane områder. Ekstreme nedbørhendelser opptrer oftere og kraftigere. Utbygging med tette flater hindrer infiltrasjon til grunnen. Den naturlige utjevningen av overvann reduseres. Dette resulterer i økende belastninger på det eksisterende avløpssystemet. Kapasiteten på avløpsnettet er mange steder overbelastet og er ikke i stand til å håndtere overvannsmengder under styrtregn. Lokal overvannsdisponering el...

  8. 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...

  9. Arctic Visiting Speakers Series (AVS)

    Science.gov (United States)

    Fox, S. E.; Griswold, J.

    2011-12-01

    The Arctic Visiting Speakers (AVS) Series funds researchers and other arctic experts to travel and share their knowledge in communities where they might not otherwise connect. Speakers cover a wide range of arctic research topics and can address a variety of audiences including K-12 students, graduate and undergraduate students, and the general public. Host applications are accepted on an on-going basis, depending on funding availability. Applications need to be submitted at least 1 month prior to the expected tour dates. Interested hosts can choose speakers from an online Speakers Bureau or invite a speaker of their choice. Preference is given to individuals and organizations to host speakers that reach a broad audience and the general public. AVS tours are encouraged to span several days, allowing ample time for interactions with faculty, students, local media, and community members. Applications for both domestic and international visits will be considered. Applications for international visits should involve participation of more than one host organization and must include either a US-based speaker or a US-based organization. This is a small but important program that educates the public about Arctic issues. There have been 27 tours since 2007 that have impacted communities across the globe including: Gatineau, Quebec Canada; St. Petersburg, Russia; Piscataway, New Jersey; Cordova, Alaska; Nuuk, Greenland; Elizabethtown, Pennsylvania; Oslo, Norway; Inari, Finland; Borgarnes, Iceland; San Francisco, California and Wolcott, Vermont to name a few. Tours have included lectures to K-12 schools, college and university students, tribal organizations, Boy Scout troops, science center and museum patrons, and the general public. There are approximately 300 attendees enjoying each AVS tour, roughly 4100 people have been reached since 2007. The expectations for each tour are extremely manageable. Hosts must submit a schedule of events and a tour summary to be posted online

  10. VISUALISERINGSTAVLA : Visualisering av Kanban boards

    OpenAIRE

    Y. F. Sam, Adam

    2013-01-01

    Hos många företag används whiteboards för att visualisera arbetsprocessen. En vanlig metodik för detta är så kallade Kanban boards, ett system av tabeller och lappar för att indikera olika avvikelser eller moment i projekt med datum och annan relevant information. Företag kan även välja att använda en mjukvara som simulerar samma metodik, ett exempel på detta är så kallade Elektroniska kanban boards. I detta arbete har båda dessa metoder (whiteboard samt en digital lösning) observerats hos tr...

  11. Effekt av ulike desinfeksjonsstrategier mot Listeria monocytogenes

    OpenAIRE

    Fossmo, Sabine

    2013-01-01

    Kontroll med bakterier som Listeria utgjør en stor utfordring for mange matprodusenter. Listeria monocytogenes er hovedsakelig et produksjonshygienisk problem, forbedret hygiene kan derfor være tiltak for å redusere overlevelse og smitteoverføring av bakterien i produksjonsmiljø. Hensikten med forsøkene i oppgaven var å undersøke effekten av ulike desinfeksjonsstrategier på drap av L. monocytogenes, både når bakteriene var i biofilm og i suspensjon. Dette inkluderte bruk av tradisjonelle desi...

  12. Efficient Software HEVC to AVS2 Transcoding

    Directory of Open Access Journals (Sweden)

    Yucong Chen

    2016-09-01

    Full Text Available The second generation of Audio and Video coding Standard (AVS is developed by the IEEE 1857 Working Group under project 1857.4 and was standardized in 2016 by the AVS Working Group of China as the new broadcasting standard AVS2. High Efficient Video Coding (HEVC is the newest global video coding standard announced in 2013. More and more codings are migrating from H.264/AVC to HEVC because of its higher compression performance. In this paper, we propose an efficient HEVC to AVS2 transcoding algorithm, which applies a multi-stage decoding information utilization framework to maximize the usage of the decoding information in the transcoding process. The proposed algorithm achieves 11×–17× speed gains over the AVS2 reference software RD 14.0 with a modest BD-rate loss of 9.6%–16.6%.

  13. Undersøkelse av forekomst av elvemusling i Grense Jakobselv

    OpenAIRE

    Aspholm, Paul Eric; Brodersen, Christopher; Nilsen, Even; Terentjev, Nikolai; Kashulin, Nikolai; Polykarpova, Natalia

    2017-01-01

    Sensommeren 2014 ble det midtre partiet av Grense Jakobselv fra Elvheim til sørenden av Lasaruskulpen undersøkt for forekomst av elvemusling. De nedre delene av Grense Jakobselv og sideelver har vært undersøkt tidligere (2003 og 2005). I disse tidligere undersøkelsene har det ikke blitt påvist elvemusling eller skall av døde muslinger. Under undersøkelsen i 22. – 23. juli 2014 ble de første elvemuslingene funnet like oppstrøms Sandvasselva. Denne undersøkelsen omfattet i alt 12 forhåndsutvalg...

  14. Fractional order absolute vibration suppression (AVS) controllers

    Science.gov (United States)

    Halevi, Yoram

    2017-04-01

    Absolute vibration suppression (AVS) is a control method for flexible structures. The first step is an accurate, infinite dimension, transfer function (TF), from actuation to measurement. This leads to the collocated, rate feedback AVS controller that in some cases completely eliminates the vibration. In case of the 1D wave equation, the TF consists of pure time delays and low order rational terms, and the AVS controller is rational. In all other cases, the TF and consequently the controller are fractional order in both the delays and the "rational parts". The paper considers stability, performance and actual implementation in such cases.

  15. Embolization of AV intra-hepatic fistulas

    Energy Technology Data Exchange (ETDEWEB)

    Mallarini, G; Saitta, S; Cariati, M; Nicorelli, M; de Caro, G

    1982-05-01

    The use of therapeutic embolization in a case of hepatic AV fistula with portal flow inversion and portal hypertension is described. Indications, technique and an illustrative case followed up for one year after the intervention are presented.

  16. Evaluering av KS’ ulike ledelsesutviklingstilbud for kommunesektoren

    OpenAIRE

    Haave, Hanne; Hafting, Tore; Haugstveit, Yngve; Odden, Sigrun

    2008-01-01

    Norsk: Denne rapporten presenterer en evaluering av ledelsesutviklingstilbud i KS. De ledelsesutviklingsprogrammene som omfattes av evalueringen er i første rekke: Rådmentor, Krefter i bevegelse, Jazz endringsledelse, Medarbeiderskap, B-link og Skreddersydde program. Evalueringens hovedproblemstilling er å undersøke om og i hvilken grad KS’ ledelsesutviklingstilbud samsvarer med dominerende perspektiver og mål i KS’ arbeidsgiverstrategi «Stolt og unik, arbeidsgiverstrateg...

  17. 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.

  18. Analyse av overvannsnettet i Ås sentrum ved bruk av simuleringsprogrammet SWMM

    OpenAIRE

    Dvergsnes, Eirunn

    2016-01-01

    Jorden står ovenfor klimaendringer med økende temperaturer og en hyppigere forekomst av intense nedbørshendelser. For overvannssystemer i urbane strøk er den økte nedbørintensiteten en stor utfordring. Urbanisering har medført en stor andel tette flater, og lukking av naturlige bekkesystemer. Dette krever en raskere bortledning av regnvannet gjennom rør i bakken. Store deler av det norske overvannssystemet er ikke dimensjonert etter dagens norske standard. Denne ble revidert se...

  19. Veileder for kravspesifikasjon for leie av kontorarealer

    DEFF Research Database (Denmark)

    Førland-Larsen, Arne

    Investorer og leietakere blir stadig mer interessert i helse- og miljøkvaliteter. Men kunnskapen er lav hos de fleste om hva de skal etterspørre og hvordan slike kvaliteter kan dokumenteres. Meglere har en viktig rolle som formidler av kvaliteter. Grønn Byggallianse har i samarbeid med blant annet...... Norsk Eiendom, Enova og NGBC utarbeidet en mal for standard kravspesifikasjon for leie av kontorlokaler. Målet med malen er å bidra til at leietager får det produktet han trenger til riktig pris og at man unngår unødig miljøbelastning. Målet er videre at å bidra og stimulere til, at innleie....../utleie prosesser startes med en dialogbasert prosess. Standarden er tenkt som et hjelpemiddel til en systematisk gjennomgang av, og diskusjon av hvilke kvaliteter som har prioritet, høy, middel eller lav prioritet for leietaker. Resultatet av dialog og diskusjon fastlegger endelige krav til kvalitet og...

  20. Å speide etter spiritualitet. En analyse av spiritualitetsbegrepet i speiderbevegelsen

    OpenAIRE

    Holmefjord, Aina

    2015-01-01

    Denne masteroppgaven inneholder analyser av speiderbevegelsens bruk av begrepet "spiritualitet" i to bøker skrevet av bevegelsens grunnlegger; "Scouting for Boys" og "Rovering to Succes" og to dokumenter av The World Organization of he Scoutmovement . Robert Baden-Powell grunnlag speiderbevegelsen i 1908 og hans litteratur og bøker publisert på tidlig 1900-tallet setter rammeverk for mye av dagens speiderbevegelses ideologi og visjon. Speiderbevegelsen har et r...

  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. Tilsetningsstoff og tekniske hjelpestoff ved produksjon av filet av hvitfisk. Faglig sluttrapport

    OpenAIRE

    Lorentzen, Grete Elisabeth

    2017-01-01

    Målsettingen med dette prosjektet har vært å øke den generelle kunnskapen om bruk av tilsetningsstoffer og tekniske hjelpestoffer ved produksjon av hvitfiskfilet. I prosjektet er det gjennomført et litteraturstudium på hvilken effekt ulike tilsetningsstoffer har på fiskefilet. Deretter er det laget en veileder hvor målet har vært å gi en lettfattet og oversiktlig informasjon om hvilke tilsetningsstoffer det er lov å bruke, hvilken effekt de har, og eksempler på praktisk bruk av tilsetningssto...

  3. Forskere i norske avisers dekning av skole

    Directory of Open Access Journals (Sweden)

    Emilia Andersson-Bakken

    2015-10-01

    Full Text Available Skolen er stadig i medienes søkelys, og i denne artikkelen ser vi nærmere på hvordan forskere bidrar med sine fagkunnskaper i skoledebatten i mediene. Den problemstillingen vi ønsker å besvare, er: Hvilken rolle har forskere i norske avisers dekning av skole? For å svare på dette har vi gjort en analyse av samtlige artikler om skole i VG, Bergens Tidende og Aftenposten i 2013. Resultatene viser at det forekommer en navngitt forsker i ca. 12 % av avisenes artikler om skole (209 av 1712 artikler. Det er imidlertid forskjell mellom avisene, og i VG opptrer det forskere i ca. 24 % av artiklene om skole, mens andelen både i Aftenposten og BT er ca. 11 %. Undersøkelsen viser videre at det er mange forskere som får mulighet til å uttale seg om skole i disse tre avisene, men det store flertallet av disse uttaler seg kun én gang. Vi fant også at forskere ofte uttaler seg om rammene for skolens undervisningsvirksomhet, og sjelden om det som foregår i klasserommene. Resultatene av vår empiriske undersøkelse stemmer i stor grad overens med hovedtendensene i tidligere studier av forskere i media, noe som indikerer at forskernes rolle i norske avisers skoledekning ikke skiller seg markant ut fra den rollen forskere har i media generelt. Våre resultater peker imidlertid på én viktig forskjell: Forskere ser ut til å være uvanlig godt synlige i norske avisers dekning av skole.Nøkkelord: skoleforskning, media, forskningsformidlingAbstractSchool is a frequently debated topic in the media, and this article investigates how researchers contribute with their knowledge in this media debate. The research question is: What characterizes researcher participation in Norwegian newspaper coverage of school? To answer this question we have analyzed all articles about school in the Norwegian newspapers VG, Bergens Tidende and Aftenposten during 2013. The results show that there is a named researcher in about 12 % of the newspaper articles about school

  4. Redesign av Escola forlag sine skoleordbøker

    OpenAIRE

    Heggen, Inger Helene

    2007-01-01

    Rapporten består av en litteraturstudie (med begrepsavklaringer og en gjennomgang av ordboktypografiens historie – med hovedvekt på engelsk ordboktypografi), en analyse av ettspråklige ordbøker i dag (både norske og engelske), og en analyse av Escola Forlags ordbøker (bokmål og nynorsk utgave). Til slutt – en demononstrasjon av min resulterende redesign av ordbøkene (omslag og innmat). Her har det vært særlig viktig å tydeliggjøre oppslagsordene og oppslagsordenes underelementer s...

  5. 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. Statens forurensningstilsyn (SFT)

  6. Distributed Trust Management and Rogue AV Software

    Science.gov (United States)

    2010-06-10

    Integrate with QTM – Particularly important in federated systems (e.g., dynamically composable SOAs) • Investigate the use of reactive mechanisms – Global...of demonstrators surfaced on Capitol Hill in opposition to the Democrats’ health care legislation. MAGAZINE PREVIEW Making Health Care Better By...sale will be sent on saving green forests in Amazonia . Have more questions? You can contact us easy via Online Supoort. Green AV an award-winning

  7. 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

  8. Design av oppbevaringsprodukt: Utvikling av et entrémøbel for IKEA

    OpenAIRE

    Bosvik, Helene

    2010-01-01

    Et av IKEAs store satsingsområder er oppbevaring. IKEA ønsker å nå ut til så mange mennesker som mulig med sine produkter. Deres visjon om å skape en bedre hverdag for de mange menneskene er et bevis på dette. Temaet for dette masterprosjektet har vært oppbevaring. En del av oppgaven har også vært å finne fokusområde innenfor temaet oppbevaring. Valget falt på entreen og utviklingen av entrémøbelet RÅDE. Oppgaven er gjort i samarbeid med IKEA Leangen i Trondheim. RÅDE er utviklet blant an...

  9. Barns opplevelse av postoperativ smerte og smertebehandling

    OpenAIRE

    Næss, Torgun

    2016-01-01

    Master i intensivsykepleie Bakgrunn: Postoperativ smerte hos barn er underbehandlet til tross for mye smerteforskning. Ulike studier har vist at barn opplever moderat til sterk smerte postoperativt. Ubehandlet smerte kan skape unødvendig lidelse for barn og øker risiko for komplikasjoner. Hensikt: Det finnes ingen forskning på norske barns opplevelse av postoperativ smerte og smertebehandling. Studiens hensikt er å få en større forståelse for hvordan barn opplever postoperativ smerte og sm...

  10. Implementering av BIM i bærekraftig oppgradering av bygninger: Mulighetsstudie for utføring av tilstandsanalyser med nettbrett

    OpenAIRE

    Vik, Vidar Samson

    2012-01-01

    Vi opplever i dag en økt hyppighet av klimakatastrofer som flom og tørke som følge av økt konsentrasjon av klimagasser i atmosfæren. Dette har ført til et behov for å redusere klimagassutslippene fra industrien på jorden. Byggebransjen, ofte kalt 40 % -bransjen, står for om lag 40 % av klimagassutslippene, og har dermed et stort potensial for reduksjon. BIM er en prosess som søker å redusere ressursbruken, og er dermed en bærekraftig prosess som har blitt tatt i bruk i stor skala i nybygg. Re...

  11. Surhetsvariasjoner som følge av nedtapping av et regulert vann.

    OpenAIRE

    Selmer-Olsen, A. R.

    1981-01-01

    Det har vært utført analyser og lagringsforsøk med prøver av tørrlagt bunnmateriale fra Trevatn tatt våren 1976. Prøver tatt ute i terrenget om høsten etter en lang tørr sommer viste stort sett samme bilde som prøvene fra våren etter lagring i laboratoriet under aerobe betingelser. Tabell 2 viser hvordan pH og SO4-S varierer med Iagringsbetingelsene. Oksydasjonsprosessene som slikt materiale blir utsatt for ved lufttilgang over et lengre tidsrom kan resultere i utvasking av meget sure forbind...

  12. Utveckling av examination av examensarbeten på kandidatnivå

    OpenAIRE

    Trofast, Tobias; Haugum, Dag; Lundberg, Jonas; Nygren, Victoria; Nyström, Tommie; Svensson, Gary; Thunborg, Maria; Törnqvist, Tomas

    2012-01-01

    Detta paper beskriver arbetet med att utveckla en modell för utveckling av examination av examensarbeten på kandidatnivå (UEX) vid Institutionen för Teknik och Naturvetenskap, Linköpings Universitet under läsåret 2010-2011. Modellen har utvecklats och testats vid utbildningsprogrammet Grafisk Design och Kommunikation (GDK), men med syfte att kunna appliceras även på andra kandidatprograms examensarbete. Det projektet prövat är om man kan höja kvaliteten på examensarbeten och effektiviteten i ...

  13. Upplevelser av "Employer Branding" : En kvalitativ fallstudie av anställda hos BA

    OpenAIRE

    Hermansson, Otto; Elisabeth, Mackenhauer

    2016-01-01

    Syftet med denna studie var att undersöka hur medarbetarna på BA (fiktivt namn) upplever organisationens interna Employer Branding-arbete samt att belysa de aspekter som eventuellt skiljer strategi mot upplevelse. Det gjordes ett målinriktat urval av organisation medan valet av deltagare var ett slumpmässigt urval vilket resulterade i åtta respondenter (n= 8). Studien var en kvalitativ fallstudie med ett psykologiskt angreppssätt och innehöll en ostrukturerad intervju som tillsammans m...

  14. AVS user's guide on the basis of practice

    International Nuclear Information System (INIS)

    Masuko, Kenji; Kato, Katsumi; Kume, Etsuo; Fujii, Minoru.

    1997-07-01

    The special guides for the use of visualization software AVS have been developed at Japan Atomic Energy Research Institute (JAERI). The purpose of these guides is to help the AVS users understand easily the use of the one, due to the fact that it is so difficult for beginners to understand the original manuals. In this report, 'Transportation Evacuation Simulation' is taken up as an object of visualization, and the procedure of visualization and images recording by using the AVS are described. By using the AVS according to this report, a series of the procedure which are necessary for use of the AVS can be acquired. (author)

  15. 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

  16. Bruk av amniocenteser og chorionbiopsier i Norge

    Directory of Open Access Journals (Sweden)

    Guttorm Haugen

    2009-10-01

    Full Text Available  SAMMENDRAGInvasiv prenatal diagnostikk i form av amniocentese (fostervannsprøve og chorionbiopsi (morkakeprøveutføres i ca. 2% av alle svangerskap i Norge per år. Dette er betydelig færre undersøkelser ennhva som utføres i de andre nordiske land. De fleste får utført amniocentese pga. høy maternell alder(aldersindikasjon som her i landet er ≥ 38 år ved fødselstermin. Chorionbiopsi er forbeholdt kvinnermed kjente arvelige lidelser i familien, dvs. kvinner med høy risiko for å få et affisert foster. De undersøkelsersom foreligger over svangerskapsutfall samt forekomst av komplikasjoner etter amniocenteseog chorionbiopsi er hovedsakelig utført i andre land på kvinner med generelt lavere risiko ( ≥ 35 år ennfor dem som får utført invasiv prenatal diagnostikk i Norge. Pga. vår restriktive praksis kan ikke disseresultatene uten videre overføres til Norge. Vi mangler eksakte data over svangerskapsutfall og evt.komplikasjoner etter disse undersøkelsene i en norsk populasjon.Haugen G, van der Hagen CB. The use of amniocentesis and chorionic villus sampling in Norway.Nor J Epidemiol ENGLISH SUMMARYAmniocentesis or chorionic villus sampling are performed in about 2% of all pregnancies in Norwaywhich is far less than in the other Nordic countries. Most of the amniocenteses are performed due toadvanced maternal age. In Norway this is defined as maternal age ≥ 38 years at term. Couples withknown chromosomal aberrations or genetic diseases in their families, i.e. women at a high risk of havingan affected fetus, are offered chorionic villus sampling. Earlier studies on complications and pregnancyoutcome following amniocentesis or chorionic villus sampling have been performed in other countriesmainly on women at a lower risk ( ≥ 35 years than for the women having such tests in Norway. We donot have data on pregnancy outcome and possible complications following amniocentesis and chorionicvillus sampling in a Norwegian

  17. Genetics Home Reference: prion disease

    Science.gov (United States)

    ... which have overlapping signs and symptoms, include familial Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), and fatal ... Sc . Sporadic forms of prion disease include sporadic Creutzfeldt-Jakob disease (sCJD), sporadic fatal insomnia (sFI), and variably protease- ...

  18. Analys av nickel med ICP-MS

    OpenAIRE

    Wallman, Karin; Löfgren, Stefan; Sonesten, Lars; Demandt, Christian

    2009-01-01

    Vid en granskning av nickelhalter för perioden 1985-2007 upptäcktes ett nivåskifte i tidsserierna på flera stationer mellan 2001 och 2002. Skiftet visade sig bero på att prover innan 2002 inte korrigerades för kalciuminterferens, medan prover under åren 2002-2007 korrigerades. Syftet med denna rapport är att utreda om resultaten före 2002 i efterhand kan korrigeras för kalciuminterferensen. Från 2002 finns ett antal prover (N=347) med resultat som både är kalciumkorrigerade och som inte är de...

  19. An Overview of the HomePlug AV2 Technology

    Directory of Open Access Journals (Sweden)

    Larry Yonge

    2013-01-01

    Full Text Available HomePlug AV2 is the solution identified by the HomePlug Alliance to achieve the improved data rate performance required by the new generation of multimedia applications without the need to install extra wires. Developed by industry-leading participants in the HomePlug AV Technical Working Group, the HomePlug AV2 technology provides Gigabit-class connection speeds over the existing AC wires within home. It is designed to meet the market demands for the full set of future in-home networking connectivity. Moreover, HomePlug AV2 guarantees backward interoperability with other HomePlug systems. In this paper, the HomePlug AV2 system architecture is introduced and the technical details of the key features at both the PHY and MAC layers are described. The HomePlug AV2 performance is assessed, through simulations reproducing real home scenarios.

  20. Avveckling av aktiebolag : Case: Likvidation av Företag X Ab

    OpenAIRE

    Kallio, Josefine

    2015-01-01

    Syftet med detta lärdomsprov är att ta reda på hur man idag i Finland kan avveckla ett aktiebolag. De fyra olika avvecklingssätten likvidation, konkurs, fusion och delning av aktiebolag är de sätt som används i Finland för att upplösa aktiebolag. I lärdomsprovet har fokus mest lagts på likvidation av aktiebolag. Lärdomsprovet ger svar på bl.a. hur en likvidation går till, vem som kan vara likvidator, vad som är skillnaden mellan en frivillig likvidation och en tvångsmässig likvidation m.m. Lä...

  1. Når kontakter betyr alt : En studie av bruken av nettverksmediet LinkedIn

    OpenAIRE

    Blaalid, Maren Hyvang

    2012-01-01

    LinkedIn er et av de mest populære sosiale mediene i Norge og tiltrekker seg stadig flere medlemmer. I denne masteroppgaven studerer jeg bruken av LinkedIn for å undersøke hva som er det særegne ved dette nettverksmediet. Jeg har utført en spørreundersøkelse blant 280 brukere for å få innsikt i hvem de er, hvorfor de bruker LinkedIn og hvordan de gjør det. Funnene fra analysen viser at brukerne kjennetegnes ved at de er selvstendige og formålsrasjonelle, som bruker LinkedIn strategisk for å n...

  2. 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.

  3. Effekt av melkesyrebakteriers metabolisme på utviklingen av Escherichia coli O157:H7 i melk

    OpenAIRE

    Westblad, Anne Margrethe

    2010-01-01

    Dagens regelverk sier at all melk som omsettes skal være varmebehandlet, men åpner samtidig for salg av melkeprodukter basert på upasteurisert melk, forutsatt overholdelse av visse krav. Dermed kan småskalavirksomheter framstille melkeprodukter av upasteurisert melk hvis de skulle ønske det. Et slikt ønske er ofte begrunnet i tradisjoner og praktiske forhold. I tillegg er det flere som påstår at råmelk er sunnere enn pasteurisert melk og at råmelkas mikroflora hemmer vekst av uønskede bakteri...

  4. EST Table: AV398539 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398539 NV021929 11/12/09 GO hit GO:0005622(intracellular)|GO:0008270(zinc ion bin...0769|gb|EEZ97216.1| hypothetical protein TcasGA2_TC011009 [Tribolium castaneum] AV398539 NV02 ...

  5. AVS-1357 inhibits melanogenesis via prolonged ERK activation.

    Science.gov (United States)

    Kim, Dong-Seok; Lee, Hyun-Kyung; Park, Seo-Hyoung; Chae, Chong Hak; Park, Kyoung-Chan

    2009-08-01

    In this study, we demonstrated that a derivative of imidazole, AVS-1357, is a novel skin-whitening compound. AVS-1357 was found to significantly inhibit melanin production in a dose-dependent manner; however, it did not directly inhibit tyrosinase. Furthermore, we found that AVS-1357 induced prolonged activation of extracellular signal-regulated kinase (ERK) and Akt, while it downregulated microphthalmia-associated transcription factor (MITF) and tyrosinase. It has been reported that the activation of ERK and/or Akt is involved in melanogenesis. Therefore, we examined the effects of AVS-1357 on melanogenesis in the absence or presence of PD98059 (a specific inhibitor of the ERK pathway) and/or LY294002 (a specific inhibitor of the Akt pathway). PD98059 dramatically increased melanogenesis, whereas LY294002 had no effect. Furthermore, PD98059 attenuated AVS-1357 induced ERK activation, as well as the downregulation of MITF and tyrosinase. These findings suggest that the effects of AVS-1357 occur via downregulation of MITF and tyrosinase, which is caused by AVS-1357-induced prolonged ERK activation. Taken together, our results indicate that AVS-1357 has the potential as a new skin whitening agent.

  6. Mineralogical Mapping of Quadrangle Av-2 (belicia) and Av-3 (caparronia) on 4 Vesta.

    Science.gov (United States)

    Stephan, K.; Frigeri, A.; Barucci, M. A.; Sunshine, J.; Jaumann, R.; Palomba, E.; Blewett, D. T.; Yingst, A.; Marchi, S.; De Sanctis, C. M.; Matz, K.-D.; Roatsch, Th.; Preusker, F.; Le Corre, L.; Reddy, V.; Russell, C. T.; Raymond, C. A.

    2012-04-01

    Since the arrival of the Dawn spacecraft at 4 Vesta on July 16, 2011 the Visible and InfraRed Imaging Spectrometer (VIR) has acquired hyperspectral images of Vesta's surface, which enable to characterize Vesta's mineralogical composition in the wavelength range from 0.25 to 5.1µm. As part of the analysis of Vesta's surface composition the science team is preparing a series of 15 quadrangle maps showing the results derived from the spectroscopic analysis of the VIR and FC color data. We present preliminary results of the spectroscopic analysis achieved for the quadrangles Av-2 (Belicia) and Av-3 (Caparronia), which show Vesta's surface between 21°N - 66° N°, 0° - 90°E and 90° - 180° E, respectively. These results are based on the analysis of the combination of the visible albedo, spectral parameters including the position, depth of the pyroxene absorptions, as well as color ratio composites using the VIR channels centering at 749nm/438nm (Red), 749nm/917nm (Green) and 438nm/749nm (Blue). Vesta's rotation axis, however, is tilted ~29° with respect to its orbital plane. Since Dawn arrived during northern winter, portions of Vesta north of ~45° N are dominated by extended shadows or have not yet been imaged due to permanent night. Thus, limited FC color or VIR hyperspectral data have been available for the quadrangles Av-2 and Av-3. The illuminated parts are dominated by a heavily-cratered northern terrain with ancient troughs and grooves and named after the prominent relatively large impact craters Belicia (~37°N/48°E) and Caparronia (~36°N/167°E). Numerous impact craters of different size, morphology, and state of surface degradation are apparent. Most spectral variations are strongly affected by the extreme illumination conditions, making the analysis of albedo variations and spectral signatures rather difficult. Their interpretation thus remains. Nevertheless, VIR spectra show clear evidence of Vesta's surface composition similar to those of HED

  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. [AVS concentrations in Xinan Creek and the influencing factors].

    Science.gov (United States)

    Liu, Xiao-Bing; Wen, Yan-Mao; Li, Feng; Wu, Chang-Hua; Duan, Zhi-Peng

    2012-07-01

    Sediment and overlying water samples were collected at 10 sampling stations at Xinan Creek, a tidal river in Pearl River Delta, and analyzed for physical and chemical characteristics as well as microbial incicators, in order to reveal the main factors dominating the spatial distribution of acid volatile sulfide (AVS). The effects of Eh, SRB OC and TS on the spatial distribution of AVS were investigated and the impact of AVS on the toxicity of heavy metals in the studied area was evaluated. The results showed that the range of AVS was 0.207-41.453 micromol x g(-1), with an average of 6.684 micromol x g(-1), which is relatively high compared to the results in other studies. The AVS value of the surface layer was higher than the bottom layer in 5 stations. The AVS values in both the surface layer and the bottom layer were highly variable, the coefficients of variation being 93.61% and 153.09% , respectively. The analytical results revealed that TS was the factor with the greatest impact on the spatial distribution of AVS, and the order was TS > OC > Eh > SRB. Potential ecological risk of heavy metals existed in 60% of the smpling stations based on the value of Sigma (SEM5-AVS), however, with the criterion of [Sigma(SEM5-AVS)]/foc, none of them had inacceptable ecological risk. Furthermore, in terms of single species of heavy metals, there was certain risk of toxic effect for all the five heavy metals (Cd, Ni, Cu, Zn and Pb). The above mentioned results will provide valuable data for the in-depth study of the formation mechanism of AVS and helpful reference for environmental impact assessment and scientific rehabilitation of heavy metals in polluted rivers.

  9. Modellering av urbane pluviale flommer ved bruk av værradar

    OpenAIRE

    Kjølseth, Tora Marie Hveem; Vatne, Ingrid

    2017-01-01

    Ekstreme nedbørhendelser med påfølgende pluviale flommer forårsaker store skader på infrastruktur og bebyggelse. Skadene fører til betydelige samfunnskostnader, og problemet er økende. Avrenningsmodeller kan benyttes for å simulere flomforløpet til en nedbørhendelse, og er et mye brukt verktøy i planlegging, prosjektering, dimensjonering og drift av overvann-systemer. Som inngangsdata i avrenningsmodeller er det vanlig å bruke konstruert nedbør, der det tas utgangspunkt i målinger og statisti...

  10. Transport og akkumulering av jern i profiler av et dyrket myrareal

    OpenAIRE

    Ødelien, M.; Selmer-Olsen, A. R.; Lie, Ole

    1980-01-01

    En mindre del av et stort dyrket myrareal på eiendommen Vivang, Våler i Solør, har uvanlig jernrik torv. Her har havre hatt god vegetativ vekst, men meget dårlig kjerneutvikling (5,10). Det siste har vist seg å skyldes molybdenmangel, som særlig må antas å ha årsakssammenheng med det store jerninnholdet i torva. I denne artikkelen er arealet med molybdenmangel kalt A og et tilgrensende areal uten synlige tegn til slik mangel B. Tabellene 1-3 viser kjemiske analyseresultater for torv fra 3 pro...

  11. En undersökning av Norwegians kriskommunikation under flygstrejken 2015. : En textanalys av Facebookinlägg och pressmeddelanden.

    OpenAIRE

    Emma, Mickelsson

    2016-01-01

    Numera lever vi i ett krissamhälle där vi nästan varje dag nås av budskap från olika typer av kriser. När en organisation drabbas av en kris är det viktigt att tänka på vilken kommunikation man använder för att nå ut med sitt budskap. Uppsatsens syfte var att undersöka hur en organisation använder sig av olika försvarsstrategier och retoriska appeller i sin kriskommunikation. Organisationen som valdes ut var flygbolaget Norwegian och deras hantering av den 11 dagar långa flygs...

  12. Slutrapport - utökad samordning av landskapsövervakning och uppföljning av Natura 2000

    OpenAIRE

    Ståhl, Göran; Gardfjell, Hans; Glimskär, Anders; Hagner, Åsa; Holm, Sören; Walheim, Mats

    2007-01-01

    I denna kortversion av 2006 års slutrapport från projektet ”utökad samordning av landskaps- övervakning och uppföljning av Natura 2000” redovisas de viktigaste resultaten på ett sätt som gör dem enklare tillgängliga för inriktningsbeslut om ambitionsnivå för uppföljnings- arbetet. Nuvarande målsättning är att Naturamoment införlivas i Nationell Inventering av Landskapet i Sverige (NILS) och Riksinventeringen av Skog (RIS) från och med 2008; under 2009 beräknas kompletteran...

  13. An Overview of the HomePlug AV2 Technology

    OpenAIRE

    Yonge, Larry; Abad, Jose; Afkhamie, Kaywan; Guerrieri, Lorenzo; Katar, Srinivas; Lioe, Hidayat; Pagani, Pascal; Riva, Raffaele; Schneider, Daniel M.; Schwager, Andreas

    2013-01-01

    HomePlug AV2 is the solution identified by the HomePlug Alliance to achieve the improved data rate performance required by the new generation of multimedia applications without the need to install extra wires. Developed by industry-leading participants in the HomePlug AV Technical Working Group, the HomePlug AV2 technology provides Gigabit-class connection speeds over the existing AC wires within home. It is designed to meet the market demands for the full set of future in-home networking co...

  14. A case of pancreatic AV malformation in an elderly man.

    Science.gov (United States)

    Gupta, Vipin; Kedia, Saurabh; Sonika, Ujjwal; Madhusudhan, Kumble Seetharama; Pal, Sujoy; Garg, Pramod

    2018-06-01

    A 60-year-old man presented with recurrent abdominal pain and weight loss for 6 months. Abdominal imaging showed a large vascular lesion in the head and neck of pancreas suggestive of arteriovenous malformation (AV malformation). Endoscopic ultrasound was done which showed features of AV malformation with no evidence of pancreatic malignancy. Surgery was planned for definitive treatment of malformation. Digital subtraction angiography with angioembolization was done prior to surgery to reduce vascularity of the lesion. He recovered after a pylorus preserving pancreaticoduodenectomy. Histopathology of the resected specimen confirmed the pancreatic AV malformation. There has been no recurrence at 2 years of follow-up.

  15. Förändring av förpackning som utökning av marknadsmixen : En studie av fem snabbrörliga konsumentvaror inom livsmedelsbranschen

    OpenAIRE

    Sandström, Carolina; Eriksson, Sofie

    2011-01-01

    Denna uppsats behandlar förändring av förpackning som en möjlig utökning av produktkategorin i Kotler & Kellers utvidgade marknadsmix. I marknadsmixen behandlas förpackningen som statisk under en varas livstid och studiens syfte var att se om förpackningsförändringar istället bör ske vid flertalet tillfällen. Genom existerande litteratur identifierades tre centrala begrepp som ansågs betydelsefulla för studien; förpackning, förändring av förpackning och differentiering. Fem olika företag ...

  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. Practically perfect: learning by doing at AVS congress.

    Science.gov (United States)

    2017-02-18

    It has been some time since Cambridge vet school last hosted the annual AVS congress, which meant that this year's congress committee faced a steep learning curve. However, as Gill Harris reports, it rose to the occasion. British Veterinary Association.

  18. Knihovna Sociologického ústavu AV ČR

    Czech Academy of Sciences Publication Activity Database

    Hesová, Nela

    2017-01-01

    Roč. 7, č. 3 (2017) E-ISSN 1805-2800 Institutional support: RVO:68378025 Keywords : library Subject RIV: AF - Documentation, Librarianship, Information Studies https://www.lib.cas.cz/casopis-informace/knihovna-sociologickeho-ustavu-av-cr/

  19. EST Table: AV399390 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399390 NV120162 11/12/09 n.h 10/09/28 100 %/181 aa ref|YP_002884244.1| Ac13-like ...yhedrovirus] 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 AV399390 NV12 ...

  20. AVS (Application Visualization System) user`s guide

    Energy Technology Data Exchange (ETDEWEB)

    Masuko, Kenji; Kato, Katsumi [Research Organization for Information Science Technology, Tokai, Ibaraki (Japan); Gorai, Kazuo; Yamazaki, Kazuhiko

    1996-03-01

    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 for image processing software and an animation processing system (video, frame scan converter). This report summarizes the information to use AVS and to develop and maintain computer and network environment for image processing. (author).

  1. 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.

  2. 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.

  3. Prion diseases of the brain; Prionenerkrankung des Gehirns

    Energy Technology Data Exchange (ETDEWEB)

    Lutz, Kira; Urbach, Horst [Universitaetsklinik Freiburg (Germany). Klinik fuer Neuroradiologie

    2015-09-15

    The prion diseases of the brain, especially Creutzfeldt-Jakob disease, are rare fatal neurodegenerative disorders. A definitive CJD diagnosis is currently only possible by a brain biopsy or post mortem autopsy. The diagnosis of Creutzfeldt-Jakob disease is based on clinical signs, pathognomonic EEG, on typical MRI findings and the examination of the cerebrospinal fluid. Using the MRI the diagnosis Creutzfeldt-Jakob disease can be confirmed or excluded with high certainty. The MRI examination should contain diffusion-weighted and FLAIR imaging sequences. This review article provides an overview of the prion diseases of the brain with the corresponding imaging findings.

  4. Prion diseases of the brain

    International Nuclear Information System (INIS)

    Lutz, Kira; Urbach, Horst

    2015-01-01

    The prion diseases of the brain, especially Creutzfeldt-Jakob disease, are rare fatal neurodegenerative disorders. A definitive CJD diagnosis is currently only possible by a brain biopsy or post mortem autopsy. The diagnosis of Creutzfeldt-Jakob disease is based on clinical signs, pathognomonic EEG, on typical MRI findings and the examination of the cerebrospinal fluid. Using the MRI the diagnosis Creutzfeldt-Jakob disease can be confirmed or excluded with high certainty. The MRI examination should contain diffusion-weighted and FLAIR imaging sequences. This review article provides an overview of the prion diseases of the brain with the corresponding imaging findings.

  5. 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|

  6. Kvantifisering av overvann: Case Brøset

    OpenAIRE

    Huurnink, Jon Egenberg

    2012-01-01

    Oppgaven forsøker å vise den systemresponsen som er særegen for konvensjonelle tiltak og blå-grønne tiltak. Dette er aktuelt på Brøset bydel som en del av Fremtiden byer prosjektet og skal bygges om til boligområde. MIKE URBAN og MIKE 21 er benyttet til å lage en konseptuell modell.Ved å sammenligne sommer- og vinterforhold, i tillegg til ulike gjentaksintervaller (1, 20 og 100år), vil kurver for videreført vannmengde gi et inntrykk av tiltakenes effekt. Dette gir beslutningsstøtte for Komm...

  7. EST Table: AV398396 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398396 NV021762 11/12/09 GO hit GO:0005622(intracellular)|GO:0008270(zinc ion bin...ding) 10/09/28 n.h 10/08/28 n.h 10/08/27 n.h 10/09/10 30 %/120 aa AGAP003111-PA Protein|2R:32505726:32508690:1|gene:AGAP003111 10/09/10 n.h 10/09/10 n.h AV398396 NV02 ...

  8. Vindkraftverk av UHPC 2.2 : En undersökning av högpresterande betong med syntetfiberarmeringen STRUX

    OpenAIRE

    Rydén, Michaéla; Nilsson, Thina

    2013-01-01

    Användandet av betong som ersättare för stål vid produktionen av vindkraftverkstorn har ökat den senaste tiden. Betongtorn är betydligt billigare än ståltorn men problem som sprickbildningar, frostsprängningar och följaktligen armeringskorrosion har uppstått bl a på grund av vibrationer från rotorn. I fundamentet i vindkraftverk kan ovan nämnda problem också uppstå och det uppfyller således inte alltid funktionskraven. Det här examensarbetet undersöker möjligheten att eliminera dessa problem ...

  9. User guide of AVS/ITBL for numerical environmental system

    International Nuclear Information System (INIS)

    Suzuki, Yoshio; Matsumoto, Nobuko; Yamagishi, Nobuhiro; Arakawa, Takuya; Kuraishi, Hideaki

    2005-02-01

    The Center for Promotion of Computational Science and Engineering of the Japan Atomic Energy Research Institute has carried out the ITBL (Information-Technology Based Laboratory) project which is one of e-Japan priority policy programs. The goal of the ITBL project is to create the vertical research environment in which intellectual resources such as remote computers, programs and data can be shared in Japanese research institutions and cooperative studies among researchers can be supported. AVS/ITBL is the visualization tool which has been developed aiming at realizing the efficient visualization in the ITBL environment. This visualization tool is one of the tools of ITBL infrastructure software and operates in cooperation with AVS/Express. Main functions of AVS/ITBL are as follows: it can directly read data files located on remote computers, it can display and control an image on the web browser, it can collaboratively display an image among remote researchers, and it can perform visualization process as a batch. In this paper, utilization of AVS/ITBL to the numerical environmental system, which is one of the applications in ITBL project, is presented. And the outline of the operation in this utilization is indicated. (author)

  10. 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

  11. 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

  12. 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

  13. 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-- ...

  14. EST Table: AV399990 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399990 br--0239 10/09/28 100 %/110 aa ref|NP_001037364.1| cellular retinoic acid .../09/10 57 %/107 aa gnl|Amel|GB15299-PA 10/09/10 59 %/107 aa gi|282165782|ref|NP_001164130.1| cellular FABP-like protein isoform 1 [Tribolium castaneum] NM_001043899 br-- ...

  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: 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-- ...

  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: 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

  19. Screening av PFAS og Dekloran forbindelser i utvalgte arktiske toppredatorer

    OpenAIRE

    Schlabach, Martin; Gabrielsen, Geir Wing; Herzke, Dorte; Hanssen, Linda; Routti, Heli; Borgen, Anders

    2017-01-01

    This report summarizes the findings of a screening study into the occurrence of selected perfluorinated compounds (PFCs) and dechloranes in Arctic top predators. The emerging PFCs F53 F53B, and PFBS were not detected neither in bird eggs nor in mammals. However, different dechloranes were detected in all samples. Screening av PFAS og Dekloran forbindelser i utvalgte arktiske toppredatorer

  20. 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-- ...

  1. 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-- ...

  2. 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)

  3. Kokken lagar mat! Utøving og utvikling av yrkeskunnskap på kjøkenet sett i lys av 2300 år gamle kunnskapsformer.

    OpenAIRE

    Gascogne, Marit

    2011-01-01

    Master i yrkespedagogikk Tema for masteroppgåva er utøving og utvikling av yrkeskunnskap i kokkfaget. Bakgrunnen for arbeidet er resultat frå andre forskingsprosjekt i masterstudiet og innføringa av den nye reforma, kunnskapsløftet, i den vidaregåande skulen. Analyse av litteratur, observasjon og intervju av yrkesutøvarar er metodane som er brukt for å finne svar på problemstillinga: Korleis kan Aristoteles sine gnosis-former brukast til å skildre yrkeskunnskap og utvikling av yrkesk...

  4. Påliteligheten av selvrapportert alkoholkonsum. Svalbardstudien 1988-89

    Directory of Open Access Journals (Sweden)

    Georg Høyer m. fl

    2009-10-01

    Full Text Available  SAMMENDRAGSvalbard er spesielt godt egnet til å vurdere påliteligheten av selvrapportert alkoholkonsum. På grunn av detlave prisnivået for alkohol er ulovlig import eller hjemmeproduksjon av alkohol ukjent på Svalbard. Dessutener samfunnet lite og oversiktlig, slik at det er praktisk mulig å undersøke hele befolkningen. I den aktuellestudien registrerte vi alt salg av alkohol til fastboende nordmenn på Svalbard i oktober og november 1988. Isamme tidsperiode ble alle nordmenn 18 år eller eldre invitert til å ta del i en helseundersøkelse der det inngikkspørsmål om alkoholbruk. Vi fant at selvrapportert alkoholkonsum utgjorde rundt 40% av salgsvolumet.På grunn av de spesielle forhold når det gjelder alkoholomsetning på Svalbard er det grunn til å anta atestimatet i denne studien er mer pålitelig sammenlignet med tilsvarende studier fra andre områder.Høyer G, Nilssen O, Brenn T, Schirmer H. The reliability of self-reported alcohol consumption.The Svalbard study 1988-89. Nor J Epidemiol 1996; 6 (1: 109-113.ENGLISH SUMMARYThe Norwegian island of Spitzbergen, Svalbard offers a unique setting for validation studies on self-reportedalcohol consumption. No counterfeit production or illegal import exists, thus making a complete registrationof all sources of alcohol possible. In this study we recorded sales from all agencies selling alcohol onSvalbard over a two month period in 1988. During the same period all adults living permanently on Svalbardwere invited to take part in a health screening. As part of the screening a self-administered questionnaire onalcohol consumption was introduced to the participants. We found that the self-reported volume accounted forapproximately 40 percent of the sales volume. Because of the unique situation applying to Svalbard, theestimate made in this study is believed to be more reliable compared to other studies using sales volume tovalidate self-reports.

  5. Hvilken rolle spiller hybrid shopping i kundereisen ved kjøp av kosmetikk?

    OpenAIRE

    Varem, Andrea; Nervik, Lene

    2017-01-01

    Temaet for denne bacheloroppgaven er hybrid shopping i kundereisen ved kjøp av kosmetikk. Etter en omfattende gjennomgang av eksisterende teori rundt kundereisen fikk vi et inntrykk av at det er et gap i forskningen knyttet til kundereisen ved kjøp av kosmetikk, på tross av størrelsen på dette markedet. Det vi fant av teori på feltet var i stor grad basert på kvantitative undersøkelser, og vi ønsker derfor å belyse kundereisen i denne konteksten gjennom et kvalitativt forskningsdesign. Med ut...

  6. Overvåking av jordboende sopp i Røsskleiva NR, Bamble 2016

    OpenAIRE

    Brandrud, Tor Erik; Dima, Bàlint

    2017-01-01

    Brandrud, T.E. & Dima, B. 2017. Overvåking av jordboende sopp i Røsskleiva NR, Bamble 2016. – NINA Kortrapport 80. 15 s. Kartlegging (start av overvåking) av habitat-spesifikke, jordboende kalksopper i nordre del av Røsskleiva NR ble gjennomført i 2016, før oppstart av skjøtselstiltak med storfébeiting. Tilsammen 27 habitat-spesifikke arter, inkludert 18 rødlistede arter ble registrert i løpet av to registreringsrunder i 2016. Funnene fordelte seg på 10 kalkbarskogsopper, 5 kalklinde-skogs...

  7. Validation of a Real-time AVS Encoder on FPGA

    Directory of Open Access Journals (Sweden)

    Qun Fang Yuan

    2014-01-01

    Full Text Available A whole I frame AVS real-time video encoder is designed and implemented on FPGA platform in this paper. The system uses the structure of the flow calculation, coupled with a dual-port RAM memory between/among the various functional modules. Reusable design and pipeline design are used to optimize various encoding module and to ensure the efficient operation of the pipeline. Through the simulation of ISE software and the verification of Xilinx Vritex-4 pro platform, it can be seen that the highest working frequency can be up to 110 MHz, meeting the requirements of the whole I frame real- time encoding of AVS in CIF resolution.

  8. Leaning av arbetspunkt för urindiagnostik

    OpenAIRE

    Romar, Arne Johannes

    2017-01-01

    Lean är en metod som ämnar förbättra arbetsprocesser, öka kvalitén och förhindra slöseri av resurser. Fler och fler företag och institutioner i västvärlden har den senaste tiden börjat implementera läran och verktygen som ursprungligen kom från Toyota och deras produktionssystem. Sjukhus och sjukhuslaboratorier är inget undantag, patienterna och provmängderna blir större samtidigt som krav på inbesparningar sker. Därför behövs alternativa lösningar i form av lean för att få tillstånd en effek...

  9. 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...

  10. Nyttan av franchising i den svenska fastighetsmäklarbranschen

    OpenAIRE

    Herke, Marie; Olivers, Marielle

    2012-01-01

    Franchising är det mest vanliga kedjekonceptet i fastighetsmäklarbranschen och flera av de största fastighetsmäklarkedjorna är franchisekedjor. De fristående mäklarbyråerna får allt svårare att klara sig i den ökade konkurrensen. Det är främst fristående mäklarbyråer på mindre orter med ett etablerat varumärke samt nischade mäklarbyråer som klarar sig i konkurrensen med de stora kedjorna. På senare år har antalet kedjeanslutna fastighetsmäklare ökat betydligt och flertalet av mäklarkedjorna ä...

  11. Utforming av stålrammer til ridehall

    OpenAIRE

    Halvorsrud, Roar

    2013-01-01

    NORSK: Oppgaven går ut på å dimensjonere forskjellige bærerammer til en ridehall på kobberud gård i Lier kommune i Buskerud. Kriterier i oppgaven er at alle forbindelser mellom søyler og bjelker skal kunne utføres som en boltet forbindelse for en så praktisk oppreising av bygget som mulig. Det gir også mulighet for og montere selve bæresystemet på egenhånd om man har nødvendig utstyr. Det er dermed viktig at man bruker så lite stål som mulig for å redusere vekt av søyler og bjelker. Bearbe...

  12. Electrical storm after CRT implantation treated by AV delay optimization.

    Science.gov (United States)

    Combes, Nicolas; Marijon, Eloi; Boveda, Serge; Albenque, Jean-Paul

    2010-02-01

    We present a case of symptomatic ischemic heart failure with an indication for cardiac resynchronization and implantable cardiac defibrillator therapy in primary prevention. After implantation, the patient developed a severe electrical storm with multiple shocks. Hemodynamic improvement based only on AV delay, guided by echocardiography and ECG, brought about a dramatic improvement in the situation. We discuss the pathophysiology of electrical storm occurring immediately after LV pacing.

  13. 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 ...

  14. 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.

  15. Symmetrical Corticobasal Syndrome Caused by a Novel c.314dup Progranulin Mutation

    NARCIS (Netherlands)

    Dopper, E.G.; Seelaar, H.; Chiu, W.Z.; de Koning, I.; van Minkelen, R.; Baker, M.C.; Rozemuller, A.J.; Rademakers, R.; van Swieten, J.C.

    2011-01-01

    Corticobasal syndrome (CBS) is characterised by asymmetrical parkinsonism and cognitive impairment. The underlying pathology varies between corticobasal degeneration, progressive supranuclear palsy, Alzheimer's disease, Creutzfeldt-Jakob disease and frontotemporal lobar degeneration sometimes in

  16. Symmetrical corticobasal syndrome caused by a novel c.314dup progranulin mutation

    NARCIS (Netherlands)

    E.G.P. Dopper (Elise); H. Seelaar (Harro); W.Z. Chiu (Wang Zheng); I. de Koning (Inge); R. van Minkelen (Rick); M.C. Baker (Matthew); A.J.M. Rozemuller (Annemieke); R. Rademakers (Rosa); J.C. van Swieten (John)

    2011-01-01

    textabstractCorticobasal syndrome (CBS) is characterised by asymmetrical parkinsonism and cognitive impairment. The underlying pathology varies between corticobasal degeneration, progressive supranuclear palsy, Alzheimer's disease, Creutzfeldt-Jakob disease and frontotemporal lobar degeneration

  17. Human prion diseases in The Netherlands : clinico-pathological, genetic and molecular aspects

    NARCIS (Netherlands)

    Jansen, C.

    2011-01-01

    Prion diseases, or transmissible spongiform encephalopathies (TSEs), are invariably fatal neurodegenerative disorders that can be sporadic, inherited or acquired by infection. In humans, TSEs comprise three major groups showing a wide phenotypic heterogeneity: Creutzfeldt-Jakob disease (CJD),

  18. ANALYSE AV SAMVARIASJON MELLOM MÅLT ARBEIDSBELASTNING (NAS) OG DEN SUBJEKTIVE OPPLEVELSEN AV ARBEIDSBELASTNINGEN (NASA-TLX) VED INTENSIVAVDELINGER

    OpenAIRE

    Graarud, Vibeke

    2015-01-01

    SAMMENDRAG Sykehus generelt og intensivavdelinger spesielt er under økende press for å kunne drive god og riktig ressursforvaltning. Dermed øker også behov for å kunne dokumentere intensiv-avdelingenes aktivitet og ressursbehov. Nursing Activities Score (NAS) blir ved flere av landets intensivavdelinger brukt som scoringsverktøy for arbeidsbelastning. Det er et verktøy som har til hensikt å kartlegge behovet for antall sykepleiere per pasient. Det er i Norge flere sykepleiere per intensivpasi...

  19. 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

  20. 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

  1. 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

  2. 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

  3. IceBridge LVIS POS/AV L1B Corrected Position and Attitude Data

    Data.gov (United States)

    National Aeronautics and Space Administration — The IceBridge LVIS POS/AV L1B Corrected Position and Attitude Data (IPPLV1B) data set contains georeferencing data from the Applanix 510 and 610 POS AV systems flown...

  4. IceBridge POS/AV L1B Corrected Position and Attitude Data

    Data.gov (United States)

    National Aeronautics and Space Administration — The IceBridge POS/AV L1B Corrected Position and Attitude (IPAPP1B) data set contains georeferencing data from the Applanix 510 POS AV system flown with the Digital...

  5. KS-LAB Kontroll av hydrometer ASTM 152H : (prosess 14-433 i hb014 og NS8005)

    OpenAIRE

    Nouri, El Hadj

    2007-01-01

    I forbindelse med kvalitetssikring av slemmeanalyser ved bruk av hydrometer har det vist seg å være behov for systematisk kontroll ved innkjøp og bruk av hydrometre. Rapporten beskriver registrerte avvik ved sammenligning av hydrometre og angir hvorledes kontroll av hydrometer skal foregå for å sikre at anvendt utstyr tilfredsstiller gjeldende krav.

  6. Et studium av samfunnsansvar (CSR) for norske utenlandsetablerte akvakulturselskaper; Marine Harvest og Cermaq Mainstream i Chile

    OpenAIRE

    Sæther, Malin

    2010-01-01

    Oppgaven er et studium av samfunnsansvar for norsk utenlandsetablert akvakulturnæring. Valg av tema er motivert av interessen for den eksisterende samfunnsdebatten omkring næringen og internasjonale næringsstrukturer med fotfeste i utviklingsland. Norsk tilstedeværelse i Chile eksemplifisert ved Marine Harvest og Cermaq Mainstream er anvendt som case studium. Presentasjon og drøfting av Corporate social responsibility (CSR), oversatt til norsk som Selskapers samfunnsansvar, ...

  7. Det afghanska valet i svensk media : En diskursanalys av svensk medias rapportering om parlamentsvalet 2010

    OpenAIRE

    Arvidsson, Jonathan

    2013-01-01

    Den här uppsatsen analyserar svensk medias rapportering om valet i Afghanistan 2010. Med hjälp av metoder framtagna av Van Dijk har en analys av artiklar som berör valet 2010 gjorts. De teoretiska utgångspunkterna är postkolonialism och orientalism. Med hjälp av de perspektiven och Van Dijks medieanalys analyseras materialet för att tydliggöra strukturer i media.

  8. 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...... for konflikt og hjelpeløshet. Helsefremmende arbeid basert på dialog og tidlig intervensjon kan forhindre at online-spilladferd kommer ut av kontroll....

  9. 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.

  10. 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...

  11. …med andre ord. ”10 stadier i oversettelse av sangtekst”. Oversettelse av Stevie Wonder og Stephen Sondheim til norsk

    OpenAIRE

    Iveland, Kari

    2017-01-01

    Norges musikkhøgskole. Masteroppgave. Utøving med fordypningsemne SAMMENDRAG AV OPPGAVEN --- Denne masteroppgaven er en del av en utøvende master og inneholder en analytisk og en praktisk del. Formålet er å finne en metode, et verktøy, for oversettelse av sangtekst, og å identifisere faktorer i språket som må tas hensyn til i oversettelse. Samlet viser den ulike språklige egenskaper som er med på å prege arbeidet med oversettelser av sangtekster. Med utgangspunkt i teorier om tolknin...

  12. 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

  13. 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.

  14. Tillämpning av IAS 40 i onoterade fastighetsbolag

    OpenAIRE

    Wallin, Fredrik; Nilsson, Karolina; Ericsson, Marina

    2006-01-01

    Enligt IAS 40 – Förvaltningsfastigheter, definieras förvaltningsfastigheter som ”mark eller byggnader eller del av byggnad som innehas i syfte att generera hyresinkomster eller värdestegring”. Onoterade fastighetsbolag i Sverige har idag möjlighet att välja mellan att värdera sina fastigheter till anskaffningsvärde eller verkligt värde. IAS – International Accounting Standards är en internationell redovisningsstandard inom EU som började tillämpas i januari 2001, men blev obligatorisk för bör...

  15. EST Table: AV404130 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available n (Reed-Steinberg cell-expressed intermediate filament-associated protein) [Tribolium castaneum] 10/08/28 53...78 aa gnl|Amel|GB30360-PB 10/09/10 54 %/199 aa gi|189241063|ref|XP_967018.2| PREDICTED: similar to restin (Reed-Steinberg cell-expres...sed intermediate filament-associated protein) [Tribolium castaneum] FS906662 pg-- ... ...AV404130 pg--0509 10/09/28 54 %/199 aa ref|XP_967018.2| PREDICTED: similar to resti

  16. Kjønnsforskjeller i forekomst av legemiddelrelaterte bivirkninger

    Directory of Open Access Journals (Sweden)

    Hedvig Nordeng

    2009-11-01

    Full Text Available  SAMMENDRAGKvinnelig kjønn er blitt identifisert som risikofaktor for legemiddelrelaterte bivirkninger både i primærhelsetjenestenog under sykehusopphold. Hyppigere forekomst av bivirkninger kan forklares delvis,men ikke fullstendig, av at kvinner som gruppe konsumerer oftere og flere legemidler. Videre har legemiddelbrukhos kvinner i mange tilfeller tilknytning til biologiske tilstander som menstruasjon, graviditetog menopause, og den øker med alderen. Det er viktig å ta i betraktning kulturelle forskjeller hoskvinner og menn som kan være med på å overestimere kjønnsforskjellene i bivirkningsforekomsten.Kvinner bruker helsevesenet hyppigere og rapporterer oftere bivirkninger. Høyere bivirkningsfrekvenshos kvinner kan også være resultat av at kvinner generelt får høyere dose per kg enn menn fordikvinner veier mindre. Kjønnsforskjeller i bivirkninger kan i tillegg være knyttet til forskjellig aktivitet ileverenzymene cytochrom P450, som metaboliserer de fleste legemidler. Strukturelle og funksjonellekjønnsforskjeller finnes også i nyrer, lunger, hjerte/kar- og mage/tarmsystemet, og påvirker i varierendegrad effekt og sikkerhet av legemidler.Nordeng H. Gender differences in the occurrence of adverse drug events.Nor J Epidemiol ENGLISH SUMMARYBoth pharmacoepidemiological and clinical studies have identified female gender as a risk factor foradverse events of drugs, both in primary care and in a hospital setting. Frequent occurrence of adverseevents can partly, but not totally, be explained by the fact that women as a group consume more drugsthan men. Women’s drug consumption can to a certain degree be related to menstruation, pregnancyand menopause, and increases with age. It is essential to take into account cultural differences that cancontribute to an overestimation of the gender effect of adverse drug events. Women use the health caresystem more frequently and report more often and more willingly adverse events. Higher

  17. Physiologic AV valvular insufficiency in cine MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Jang, Yoon Hyung; Kang, Eun Joo; Baik, Seung Kug; Ahn, Woo Hyun; Choi, Han Yong; Kim, Bong Gi [Wallace Memorial Baptist Hospital, Pusan (Korea, Republic of)

    1994-05-15

    To give a help in the interpretation of cardiac cine-MR examination, the extent, shape, and timing of appearance of signal void regions near atrioventricular(A-V) valve prospectively evaluate in the healthy population. Using an axial gradient-echo technique with small flip angle, repetition time(TR) of 36 msec and echo time(TE) of 22 msec, 20 volunteers without known valvular abnormalities undertook cardiac cine-MR imaging including atrioventricular valve areas. Transient signal void was observed within the near the tricuspid(13/20 = 65%) and mitral valves(9/20 = 45%), respectively, which is so called {sup p}hysioloic atrioventricular valvular insufficiency{sup .} Eight subjects revealed the signal void areas near both tricuspid and mitral valves but, 5 subjects did not show any evidence of physiologic insufficiency. This physiologic condition does not extend more than 1 cm proximal to A-V valve plane and is generally observed only during early systole. Its morphology is semilunar or triangular configuration with the base to the valve plane in most cases of normal tricuspid insufficiency and small globular appearance in most cases of normal mitral insufficiency. Awareness of normal signal void areas near the A-V valve and their characteristics is critical in the interpretation of cardiac cine MR examinations and maybe helpful in the study of the normal cardiac physiology.

  18. Physiologic AV valvular insufficiency in cine MR imaging

    International Nuclear Information System (INIS)

    Jang, Yoon Hyung; Kang, Eun Joo; Baik, Seung Kug; Ahn, Woo Hyun; Choi, Han Yong; Kim, Bong Gi

    1994-01-01

    To give a help in the interpretation of cardiac cine-MR examination, the extent, shape, and timing of appearance of signal void regions near atrioventricular(A-V) valve prospectively evaluate in the healthy population. Using an axial gradient-echo technique with small flip angle, repetition time(TR) of 36 msec and echo time(TE) of 22 msec, 20 volunteers without known valvular abnormalities undertook cardiac cine-MR imaging including atrioventricular valve areas. Transient signal void was observed within the near the tricuspid(13/20 = 65%) and mitral valves(9/20 = 45%), respectively, which is so called p hysioloic atrioventricular valvular insufficiency . Eight subjects revealed the signal void areas near both tricuspid and mitral valves but, 5 subjects did not show any evidence of physiologic insufficiency. This physiologic condition does not extend more than 1 cm proximal to A-V valve plane and is generally observed only during early systole. Its morphology is semilunar or triangular configuration with the base to the valve plane in most cases of normal tricuspid insufficiency and small globular appearance in most cases of normal mitral insufficiency. Awareness of normal signal void areas near the A-V valve and their characteristics is critical in the interpretation of cardiac cine MR examinations and maybe helpful in the study of the normal cardiac physiology

  19. UNIPASS for AvSP? A Broader View

    Science.gov (United States)

    Wu, N. Eva

    2001-01-01

    UNIPASS is a general-purpose probabilistic computer program consisting of three major modules, including preprocessor, solver and postprocessor. UNIPASS contains a user-friendly Graphical User Interface (GUI), numerous state-of-the-art probabilistic analysis techniques, a large library of statistical distributions and a function module with a large library of support functions that can easily define any complex limit-state function in a scripting FORTRAN-like syntax format. Its inverse probability analysis and sensitivities analysis capabilities make it a powerful design aid in any product cycle. Its precise numerical analysis engine is accurate enough to push the failure probabilities of a design to well below 10 (exp -50). UNIPASS is equipped with advanced artificial intelligence that is designed to handle systems with an essentially unlimited number of random variables with ease and efficiency. Its modular arrangement allows you to tailor an analysis to the desired level of accuracy and efficiency. The depth and comprehensiveness of UNIPASS are built upon the decades of experience and expertise of industry leaders including Boeing Aircraft, NASA and the DoD. Its rich content also makes UNIPASS a valuable instructional tool for random processes and probabilistic mechanics. The topics include: 1) Reliability in AvSP; 2) Role of UNIPASS in AvSP; and 3) Examples. This paper is in viewgraph form.

  20. Cerebral av angiomas: 3-dimensional demonstration by spiral CT

    International Nuclear Information System (INIS)

    Rieger, J.; Hosten, N.; Neumann, K.; Lemke, A.J.; Langer, R.; Lanksch, W.R.; Pfeifer, K.J.; Felix, R.

    1994-01-01

    In 20 patients with known or suspected supratentorial arteriovenous malformations, an attempt was made to see how far CT angiography with 3-dimensional reconstructions is able to make a diagnosis and to differentiate the various components of the angioma. Spiral CT was performed following an intravenous bolus injection of 60-80 ml of iodine containing contrast medium. In all patients the diagnosis was confirmed by intra-arterial DSA of the vertebral vessels. In 13 patients, av malformations could be diagnosed following multiplanar 3-D reconstructions which agreed with the findings on DSA. The large supplying vessels, the nidus and the large draining veins could be defined with certainty. In 6 patients follow-up examination after embolisation was performed. The results could be demonstrated in three dimensions and the success of treatment could be documented unequivocally. CT angiography with 3-D reconstruction is able to supply important information in the majority of intracranial av malformations, both during initial investigation and following treatment. (orig.) [de

  1. Selvbestemmelse og oppdragelse : en teoretisk analyse av oppdragelsens påvirkning på utviklingen av selvbestemmelse

    OpenAIRE

    Hagen, Marit Svennevig; Haugstvedt, Merete Lehne Rugdal

    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...

  2. Are acid volatile sulfides (AVS) important trace metals sinks in semi-arid mangroves?

    Science.gov (United States)

    Queiroz, Hermano Melo; Nóbrega, Gabriel Nuto; Otero, Xose L; Ferreira, Tiago Osório

    2018-01-01

    Acid-volatile sulfides (AVS) formation and its role on trace metals bioavailability were studied in semi-arid mangroves. The semi-arid climatic conditions at the studied sites, marked by low rainfall and high evapotranspiration rates, clearly limited the AVS formation (AVS contents varied from 0.10 to 2.34μmolg -1 ) by favoring oxic conditions (Eh>+350mV). The AVS contents were strongly correlated with reactive iron and organic carbon (r=0.84; r=0.83 respectively), evidencing their dominant role for AVS formation under semi-arid conditions. On the other hand, the recorded ΣSEM/AVS values remained >1 evidencing a little control of AVS over the bioavailability of trace metals and, thus, its minor role as a sink for toxic metals. Copyright © 2017 Elsevier Ltd. All rights reserved.

  3. O tornar-se avó no processo de individuação

    Directory of Open Access Journals (Sweden)

    Caroline Dal Ri Kipper

    Full Text Available O tornar-se avó assinala um período de transição no ciclo de vida familiar, marcado por transformações psíquicas significativas para os avós, caracterizando a quarta individuação. Este trabalho teve como objetivo investigar a experiência de tornar-se avó e sua importância no processo de individuação. Foi utilizado delineamento de estudo de caso coletivo. Onze avós maternas, com idades entre 49 e 66 anos, cujas filhas tiveram seu primeiro filho, responderam a uma entrevista semi-estruturada. Todas as avós tinham tido seus primeiros netos e a entrevista referia-se a sua experiência como avós desses netos. Os dados mostraram que o ser avó é uma fonte de renovação e renascimento. O estudo propiciou que as participantes refletissem sobre seus diferentes papéis familiares: avó, mãe, neta e filha. Os dados sugerem que tornar-se avó possibilita que antigos conflitos sejam repensados, renovando antigos vínculos e desejos, o que permite que a avó dê mais um passo rumo à sua individuação.

  4. American AV: Edgar Dale and the Information Age Classroom.

    Science.gov (United States)

    Acland, Charles R

    2017-01-01

    This article demonstrates how the influential scholar Edgar Dale, alongside a generation of educational technologists, helped build an essential place for AV materials and pedagogical methods in the American classroom. It also shows that, for decades, the Payne Fund philanthropy supported multimedia research agendas that shaped ideas about teaching and technology, far beyond involvement in their famed studies on motion pictures and children in the 1930s. With his writings and research programs, Dale advanced concepts of media experience and systematicity, which came to be understood as common sense to the information society. In so doing he was a leading contributor to the discursive and ideological structure of our age of technological and informational abundance.

  5. EST Table: AV399395 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399395 NV120168 10/09/28 100 %/139 aa ref|NP_001037386.1| glyceraldehyde-3-phosph...ate dehydrogenase [Bombyx mori] gb|ABA43638.2| glyceraldehyde-3-phosphate dehydrogenase [Bombyx mori] 10/08/28 81 %/139...id:CAA88697.1 10/09/10 89 %/142 aa AGAP009623-PA Protein|3R:37154051:37155049:1|gene:AGAP009623 10/09/10 79 %/139... aa gnl|Amel|GB14798-PA 10/09/10 84 %/139 aa gi|91088023|ref|XP_974181.1| PREDICTED: similar to glyceraldehyde 3-phosphate dehydrogenase [Tribolium castaneum] DN237090 NV12 ...

  6. Finansiering av investeringar i immateriella tillgångar

    OpenAIRE

    Voltaire, Christian; Gillebrink, Valerie

    2008-01-01

    Vid en bedömning av ett kreditärende ser banken till projektets företagsekonomiska förutsättningar. För företag som har immateriella tillgångar som de viktigaste tillgångarna i sin redovisning kan det uppstå problem när de ansöker om kredit hos banker. Detta beror på att immateriella tillgångar inte fungerar på samma sätt som materiella och därigenom tillbringar vag säkerhet för en beviljad kredit. Syftet med denna uppsats är att söka finna vad det är som krävs för att ett tjänsteföretag ska ...

  7. UTVÄRDERING AV UTVALDA SD-WAN PRODUKTER

    OpenAIRE

    Åkerblom, Jacob

    2016-01-01

    Denna jämförelsestudie ämnar att undersöka marknadens leverantörer av nätverksutrustning de- finierade som Software Defined Wide Area Networks(SD-WAN). SD-WAN är en innovativ teknik som applicerar virtualisering och Software defined networking koncept i Wide Area Networks (WAN) för att skapa kostnadseffektiva nätverk. Studien har utförts på företaget Curitiba som idag arbetar med konsulttjänster inom utvalda WAN-optimering och SD-WAN-produkter. Arbetets syfte är att utvärdera SD-WAN-lösningar...

  8. Verksamhetsanalys, Planering och Utveckling av en webbtjänst

    OpenAIRE

    Kindstedt, Tomas

    2013-01-01

    Arbetet behandlar utvecklingsprocessen av programmet SECTMA, ett cirkeltränings-program designat för människor som utövar kampsporter. Programmet är designat för alla konditionsnivåer från toppidrottare till de som endast vill förbättra sin kondition. Arbetet är tudelat, i den första delen går jag igenom teori för STOF och Agile för att skapa en grund och en förståelse för hur jag vill utveckla programmet. Den andra delen följer med utvecklingsprocessen stegvis. STOF erbjuder ett ramverk...

  9. 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.

  10. TIME VARIATION OF AV AND RV FOR TYPE Ia SUPERNOVAE BEHIND INTERSTELLAR DUST

    Science.gov (United States)

    Huang, Xiaosheng; Biederman, M.; Herger, B.; Aldering, G. S.

    2014-01-01

    TIME VARIATION OF AV AND RV FOR TYPE Ia SUPERNOVAE BEHIND NON-UNIFORM INTERSTELLAR DUST ABSTRACT We investigate the time variation of the visual extinction, AV, and the total-to-selective extinction ratio, RV, resulting from interstellar dust in front of an expanding photospheric disk of a type Ia supernova (SN Ia). We simulate interstellar dust clouds according to a power law power spectrum and produce extinction maps that either follow a pseudo-Gaussian distribution or a lognormal distribution. The RV maps are produced through a correlation between AV and RV. With maps of AV and RV generated in each case (pseudo-Gaussian and lognormal), we then compute the effective AV and RV for a SN as its photospheric disk expands behind the dust screen. We find for a small percentage of SNe the AV and RV values can vary by a large factor from day to day in the first 40 days after explosion.

  11. Sampling method, storage and pretreatment of sediment affect AVS concentrations with consequences for bioassay responses.

    Science.gov (United States)

    De Lange, H J; Van Griethuysen, C; Koelmans, A A

    2008-01-01

    Sediment treatment and sediment storage may alter sediment toxicity, and consequently biotic response. Purpose of our study was to combine these three aspects (treatment-toxicity-biotic response) in one integrated approach. We used Acid Volatile Sulfide (AVS) concentrations as a proxy of the disturbance of the sediment. AVS and Simultaneously Extracted Metal (SEM) concentrations were compared to bioassay responses with the freshwater benthic macroinvertebrate Asellus aquaticus. Storage conditions and sediment treatment affected AVS but not SEM levels. AVS can be used as a proxy for sediment disturbance. The best way to pretreat the sediment for use in a bioassay in order to maintain initial AVS conditions was to sample the sediment with an Ekman grab, immediately store it in a jar without headspace, and freeze it as soon as possible. In a survey using seven different sediments, bioassay responses of A. aquaticus were correlated with SEM and AVS characteristics.

  12. Rommet som den tredje pedagog - En studie av pedagogers forståelse av rommets betydning - med fokus på estetisk virksomhet

    OpenAIRE

    Krokstad, Inger Elisabeth

    2014-01-01

    Masteroppgaven «Rommet som den tredje pedagog i tre Reggio Emilia-inspirerte barnehager. En studie av pedagogers forståelse av rommets betydning –med fokus på estetisk virksomhet» har til hensikt å bidra til økt kunnskap om estetisk virksomhet som er forbundet med Reggio Emilias tanke om rommet som den tredje pedagog. Avhandlingens problemstilling er: Hvilke mønster kan identifiseres i barnehagepedagogens forståelse av rommet som den tredje pedagog-med fokus på estetisk virksomhet? ...

  13. Rehabilitering efter dekompression och diskoperation - framställandet av en fysioterapeutisk patientanvisning : Ett beställningsarbete av Tölö sjukhus

    OpenAIRE

    Nyberg, Jasmina

    2016-01-01

    Examensarbetet är ett praktiskt inriktat examensarbete med fokus på rehabiliteringen efter en dekompressions- och diskoperation. Det är ett beställningsarbete av Tölö sjukhus med syftet att framställa en fysioterapeutisk patientanvisning. Önskemål från uppdragsgivare var att utforma en tydlig patientanvisning för ryggopererade bestående av skriftliga instruktioner angående postoperativ rehabilitering i form av vardagliga aktiviteter, fysisk aktivitet, ryggens viloställningar, vi...

  14. Vad vill kvinnor ha? : En undersökning av en lokaltidnings bilaga i form av ett livsstilsmagasin.

    OpenAIRE

    Heurling, Åsa; Lovisa, Lesse

    2011-01-01

    Folkbladet är en dagstidning i Norrköping med en stark lokal prägel vars prenumeranter till största delen är sportintresserade män. För att försöka bredda sin läsekrets har man sedan 2009 gett ut en tidning kallad Trend, som vänder sig till kvinnor i åldern 20 - 50 år. Denna tidning med lokal prägel, i form av ett livsstilsmagasin för kvinnor, handlar om mode, skönhet, heminredning och resor. Maga- sinet ges ut i fyra nummer per år och delas ut gratis till alla hushåll i Norrköping med omnejd...

  15. Dialogmøte om teknologi for trenging av fisk i merd

    OpenAIRE

    Henriksen, Kristian; Sunde, Leif Magne; Digre, Hanne; Svendsen, Erik; Rundtop, Per; Erikson, Ulf Gøran

    2013-01-01

    SINTEF Fiskeri og havbruk arrangerte den 19. mars 2013, i samarbeid med Fiskeri- og havbruksnæringens forskningsfond (FHF), et dialogmøte med fokus på teknologi for trenging av fisk i merd. Under møtet ble status for gjeldene teknologi og operasjoner for trenging av fisk i merd, samt resultater fra forskningsprosjekter tilknyttet trengeprosessen, presentert. Videre ble det samlet innspill om utfordringer tilknyttet trenging av fisk i produksjons- og ventemerd fra oppdrettsbedrifter, leverandø...

  16. "Dem vil at barna skal ha noe å gjøre" : En casestudie av Habbo.no

    OpenAIRE

    Kvaale, Torkel

    2012-01-01

    Denne oppgaven er en kvalitativ casestudie av Habbo.no, en virtuell verden der hovedgruppen av brukere er barn og unge. Det kvalitative forskningsintervju representerer metodeverktøyet som er brukt for innsamlingen av data, og datamaterialet består av 10 intervjuer med tilsammen 13 brukere av Habbo.no, i alderen 10-12 år. Habbo ble skapt av det finske selskapet Sulake i 2000 som hevder at denne er verdens største virtuelle verden for tenåringer. Habbo.no er kun én av til sammen 35 unike H...

  17. Lyden av de mørke sjeler - Lyd og musikk i videospillet Dark Souls

    OpenAIRE

    Burdal, Kristin Johnsrud

    2014-01-01

    Dark Souls er et videospill som skiller seg fra måten de fleste moderne videospill er bygget opp. Lyd og musikk er en viktig del av dette, og i denne oppgava har vi sett på hvordan lyd og musikk brukes i videospillet. Målet var å finne ut hva lyd og musikk har å si for spillopplevelsen. Dette har vi kommet fram til ved analyse av lyden, og analyse av et utvalg av musikkstykker. For å peke på hvordan lydbruken i Dark Souls skiller seg ut, har vi i tillegg sammenligna det med lydbruken andre vi...

  18. Magnetic hard disks for audio-visual use; AV yo jiki disk baitai

    Energy Technology Data Exchange (ETDEWEB)

    Tei, Y.; Sakaguchi, S.; Uwazumi, H. [Fuji Electric Co. Ltd., Tokyo (Japan)

    1999-11-10

    Computers, consumer, and communications are converging and fusing. The key device in homes in the near future will be an audiovisual hard disk drive (AV-HDD). The reason is that there is no other AV cash memory with high capacity, high speed, and a low price than the HDD. Fuji Electric has early started developing an AV magnetic hard disk, a core-functional element of the AV-HDD, to take the initiative in the market. This paper describes the state of plastic medium development, which is regarded as a next-generation strategic commodity. (author)

  19. Effekt av å benytte sosiale medier i kampanjer for å fremme sunne levevaner

    OpenAIRE

    Strømme, Hilde

    2014-01-01

    Hensikten med denne systematiske oversikten er å svare på spørsmålet: Hva er effekten av å benytte sosiale medier i kampanjer for å påvirke holdninger, kunnskap og atferd knyttet til sunne levevaner? Metode: Systematiske søk ble gjort i 11 bibliografiske baser. Tilleggssøk ble gjort i Google Scholar. Utvelgelse og vurdering av publikasjoner ble gjort av to personer uavhengig av hverandre. Risiko for systematiske feil ble vurdert med sjekklisten til Cochrane Effective Practice and Organisation...

  20. Open-Loop Audio-Visual Stimulation (AVS): A Useful Tool for Management of Insomnia?

    Science.gov (United States)

    Tang, Hsin-Yi Jean; Riegel, Barbara; McCurry, Susan M; Vitiello, Michael V

    2016-03-01

    Audio Visual Stimulation (AVS), a form of neurofeedback, is a non-pharmacological intervention that has been used for both performance enhancement and symptom management. We review the history of AVS, its two sub-types (close- and open-loop), and discuss its clinical implications. We also describe a promising new application of AVS to improve sleep, and potentially decrease pain. AVS research can be traced back to the late 1800s. AVS's efficacy has been demonstrated for both performance enhancement and symptom management. Although AVS is commonly used in clinical settings, there is limited literature evaluating clinical outcomes and mechanisms of action. One of the challenges to AVS research is the lack of standardized terms, which makes systematic review and literature consolidation difficult. Future studies using AVS as an intervention should; (1) use operational definitions that are consistent with the existing literature, such as AVS, Audio-visual Entrainment, or Light and Sound Stimulation, (2) provide a clear rationale for the chosen training frequency modality, (3) use a randomized controlled design, and (4) follow the Consolidated Standards of Reporting Trials and/or related guidelines when disseminating results.

  1. Inventering av Suldalslågen. Produksjonspotensial for sjøvandrende laksefisk

    OpenAIRE

    Foldvik, Anders; Pettersen, Oskar

    2017-01-01

    Foldvik, A. & Pettersen, O. 2017. Inventering av Suldalslågen. Produksjonspotensial for sjøvandrende laksefisk. - NINA Kortrapport 75, 18 s. Reguleringen av Suldalslågen til kraftproduksjon har hatt negative effekter for habitat for laksefisk, blant annet i form av sedimentering og begroing av substratet. Disse prosessene har blitt forsøkt motvirket ved å ha en serie med spyleflommer på over 200 m3/s om høsten. På oppdrag fra Statkraft inverterte NINA oppvekst- og gyteforhold for laks i Su...

  2. IAS 40 i ljuset av ägarförhållanden : Värdering av förvaltningsfastigheter i börsnoterade respektive statliga bolag

    OpenAIRE

    Klarin, Olof; Sandell, Johan

    2010-01-01

    Sammanfattning: Redovisningen av förvaltningsfastigheter enligt IAS 40 har tidigare beskrivits i ett stort antal olika vetenskapliga alster. Merparten av dessa har dock tittat på börsnoterade bolag men det finns även andra företag som tillämpar IAS-reglerna. En grupp företag som gör det är de bolag som ägs av svenska staten. Ett av målen med IFRS är att skapa jämförbarhet och då bör inte ägarformen ha någon betydelse för redovisning och värdering. Denna studies syfte är därför att skapa insik...

  3. Idag lever Kjell med Britt-Marie : En kritisk diskursanalys av framställningen av homosexualitet i TV-reklam

    OpenAIRE

    Kristensson, Sophia; Olsson, Emma

    2016-01-01

    Studiens syfte är att undersöka hur homosexualitet skildras i svensk TV-reklam. Detta med anledning av mediers möjligheter till att påverka publiken samt utifrån heteronormen i samhället. För att uppfylla målet med studien har ett par frågeställningar formulerats: “Vilka diskursiva teman framkommer i resultatet och vad säger de om framställningen av homosexualitet i TV-reklam?” och “Skiljer sig framställningen av homosexualitet från framställningen av heterosexualitet och i så fall hur?”. Sex...

  4. ADVANCED VITRIFICATION SYSTEM (RIC AVS) RESEARCH AND DEVELOPMENT PROJECT

    Energy Technology Data Exchange (ETDEWEB)

    J.R. Powell; M. Reich

    2003-06-30

    The objective of this AVS testing program is to use bench-scale test equipment to produce a vitrified product at maximum waste loading from the specified AZ-101 waste simulant and conduct a TTT analysis using laboratory scale melts to show compliance with the DOE Waste Acceptance Product Specifications for Vitrified High-Level Waste Forms (WAPS). The vitrified product complies with the following WAPS. A borosilicate glass with a waste loading of 60.9-wt% was produced from a slurry feed of AZ101 simulant. Glass durability testing, glass characterization testing, and testing methodology were performed in accordance with the Department of Energy approved Test Plan. The glass has two crystalline phases and good uniformity of composition. The Product Consistency Test on the 6 location-specific samples are at least 1 to 2 orders of magnitude below the mean PCT results for the EA glass. Standard deviations were less than 10% of measured values. The glass transition temperature averaged 658 {+-} 9 C. A TTT diagram was produced. There was measured cesium loss of about 2%, and compliance with the Universal Treatment Standards.

  5. ADVANCED VITRIFICATION SYSTEM (RIC AVS) RESEARCH AND DEVELOPMENT PROJECT

    International Nuclear Information System (INIS)

    Powell, J.R.; Reich, M.

    2003-01-01

    The objective of this AVS testing program is to use bench-scale test equipment to produce a vitrified product at maximum waste loading from the specified AZ-101 waste simulant and conduct a TTT analysis using laboratory scale melts to show compliance with the DOE Waste Acceptance Product Specifications for Vitrified High-Level Waste Forms (WAPS). The vitrified product complies with the following WAPS. A borosilicate glass with a waste loading of 60.9-wt% was produced from a slurry feed of AZ101 simulant. Glass durability testing, glass characterization testing, and testing methodology were performed in accordance with the Department of Energy approved Test Plan. The glass has two crystalline phases and good uniformity of composition. The Product Consistency Test on the 6 location-specific samples are at least 1 to 2 orders of magnitude below the mean PCT results for the EA glass. Standard deviations were less than 10% of measured values. The glass transition temperature averaged 658 ± 9 C. A TTT diagram was produced. There was measured cesium loss of about 2%, and compliance with the Universal Treatment Standards

  6. Geologic and Mineralogic Mapping of Av-6 (Gegania) and Av-7 (Lucaria) Quadrangles of Asteroid 4 Vesta

    Science.gov (United States)

    Nathues, A.; Le Corre, L.; Reddy, V.; De Sanctis, M. C.; Williams, D. A.; Garry, W. B.; Yingst, R. A.; Jaumann, R.; Ammannito, E.; Capaccioni, F.; Preusker, F.; Palomba, E.; Roatsch, T.; Tosi, F.; Zambon, F.; Pieters, C. M.; Russell, C. T.; Raymond, C. A.

    2012-04-01

    NASA's Dawn spacecraft arrived at the asteroid 4 Vesta in July 2011 and is now collecting imaging and spectroscopic data during its one-year orbital mission. The maps we present are based on information obtained by the Visible and Infrared Mapping Spectrometer VIR-MS and the multi-color Framing Camera FC. VIR covers the wavelength range between 0.25 to 5.1 µm while FC covers the range 0.4 to 1.0 µm. The VIR instrument has a significant higher spectral resolution than FC but the latter achieves higher spatial resolution data. As part of the geological and mineralogical analysis of the surface, a series of 15 quadrangles have been defined covering the entire surface of Vesta. We report about the mapping results of quadrangle Av-6 (Gegania) and Av-7 (Lucaria). The Gegania quadrangle is dominated by old craters showing no ejecta blankets and rays while several small fresh craters do. The most obvious geologic features are a set of equatorial troughs, a group of three ghost craters of similar diameter (~57 km), an ejecta mantling of the Gegania crater and three smaller craters showing bright and dark ejecta rays. The quadrangle contains two main geologic units: 1) the northern cratered trough terrain and 2) the equatorial ridge and trough terrain. The quadrangle shows moderate variation in Band II center wavelength and Band II depth. FC color ratio variations of some recent craters and their ejecta are linked to the bright and dark material. The bright material is possibly excavated eucritic material while the dark material could be remnants of a CM2 impator(s) or an excavated subsurface layer of endogenic origin. The most prominent geologic features in the Lucaria quadrangle are the 40 km long hill Lucaria Tholus, a set of equatorial troughs, some relatively fresh craters with bright and dark material and mass wasting. The quadrangle contains three main geologic units: 1) the northern cratered trough terrain, 2) the equatorial ridge and trough terrain, and 3) the

  7. Elevers oppfatning av lærers tilbakemeldingspraksis: om sammenheng mellom graden av VfL-praksis og elevenes opplevde nytte av lærers skriftlige tilbakemeldinger i skriftlig norsk

    Directory of Open Access Journals (Sweden)

    Harald Eriksen

    2017-11-01

    Full Text Available Formålet med denne studien var å undersøke elevers oppfatninger av norsklærerens tilbakemeldingspraksis i skriftlig norsk. På bakgrunn av omfattende internasjonal forskning innen feltet ‘Vurdering for læring’ (VfL er det i denne studien utviklet teoridrevne hypoteser som testes ved å analysere et datasett fra to videregående skoler. Hypotesene er at elevene oppfatter lærers tilbakemelding som mer nyttig hvis den 1 retter seg mot målet med opplæringen, 2 gir elevene informasjon via en forklaring eller instruks om hvordan de skal komme nærmere målet og 3 at lærer legger til rette for bruk av tilbakemeldingen. Strukturell likningsmodellering (SEM av resultater fra en spørreundersøkelse blant elever (N = 213 er brukt som metode til å estimere forholdet mellom de fire latente variablene kriterier, forklaring, bruk og nytte (avhengig variabel. SEM er brukt fordi metoden egner seg til å undersøke sammenhenger i komplekse datasett. Funn er at elevene oppfatter at hvis lærer forklarer hvordan de kan forbedre seg, er dette positivt statistisk assosiert med opplevd nytte. Et mer overraskende funn er at lærerens tilrettelegging for bruk av tilbakemeldinger ikke er signifikant assosiert med opplevd nytte. Resultatene blir diskutert i artikkelen.

  8. 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)

  9. Association of temporary complete AV block and junctional ectopic tachycardia after surgery for congenital heart disease

    International Nuclear Information System (INIS)

    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 < 0.001), whereas no case of postoperative JET was reported in patients with permanent 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

  10. 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

  11. 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) ...

  12. Temporal dynamics of AVS and SEM in sediment of shallow freshwater floodplain lakes

    NARCIS (Netherlands)

    Griethuysen, van C.; Lange, de H.J.; Heuij, van der M.; Bies, S.C.; Gillissen, F.; Koelmans, A.A.

    2006-01-01

    Acid volatile sulfide (AVS) is an operationally defined sulfide fraction, which is considered important for trace metal fate in reduced sediments. Understanding AVS formation rates is important for the management of metal polluted sediment. However, little lis known about the fate and dynamics of

  13. 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) ...

  14. Examination of office visit patient preferences for the after-visit summary (AVS).

    Science.gov (United States)

    Neuberger, Marolee; Dontje, Katherine; Holzman, Greg; Corser, Bill; Keskimaki, Abigail; Chant, Ericka

    2014-01-01

    The federal government advocates the practice of routinely providing an after-visit summary (AVS) to patients after each office-based visit as an element of stage 1 meaningful use. A significant potential benefit of the AVS is improved patient engagement achieved by enabling patients and family members to better understand and retain key health information. The methodology for this study was a mixed-methods pilot study to examine, through the perspectives of adult primary care patients, how relevant and actionable data can be better formatted in the AVS. Results of this study suggest that the goal of the AVS to serve as a communication tool to engage and support patients is frequently not being met. Further study is needed to understand, from the viewpoints of patients and providers, what barriers are keeping them from optimally providing and using the information on the AVS.

  15. En kartlegging av forekomsten av forskjellige rusmidler blant norske motorvognførere pågrepet av politiet, med mistanke om påvirket kjøring

    Directory of Open Access Journals (Sweden)

    Kari-Mette Beylich m. fl Beylich m. fl

    2009-10-01

    Full Text Available  SAMMENDRAGSjåfører som mistenkes for påvirket kjøring blir i første omgang vurdert av politiet med henblikk på om det kunskal rekvireres alkoholanalyse eller om det også skal tas prøve med hensyn på andre rusmidler (narkotika,medikamenter med ruspotensial ved Statens rettstoksikologiske institutt (SRI. Rutinemessig vil SRI undersøkedet politiet rekvirerer, men prøvene lagres inntil to år etter at de er analysert. Ved supplerende analyse i ettertidkan man således få et mer fullstendig bilde av hva mistenkte, påvirkede bilførere hadde i kroppen da de avgaprøve. I den foreliggende undersøkelsen ble 1197 prøver fra februar og september 1993 analysert på et bredtspekter av andre rusmidler foruten alkohol, uavhengig av hva politiets mistanke hadde vært. Vi fant da at alkoholalene var til stede i 55 % av prøvene, andre rusmidler alene i 16%, både alkohol og andre rusmidler i 14%, mensverken alkohol eller andre rusmidler ble påvist i 15% av prøvene. Politiets mistanke med hensyn til alkoholpåvirkningble bekreftet av analyseresultatene i 71% av tilfellene, og mistanke om forekomst av andre rusmidler i58% av tilfellene. Tidligere studier og denne undersøkelsen viste at bilførere som mistenkes for påvirket kjøringsynes å representere en gruppe med storbruk av alkohol og misbruk av andre stoff.Beylich K-M, Christophersen AS, Skurtveit S, Bjørneboe A, Mørland J. Frequency of different drugs in  ENGLISH SUMMARYDrivers apprehended under the suspicion of drunken or drugged driving are primarily evaluated by the police withregard to whether alcohol or drugs should be looked for in the blood samples taken shortly after apprehension.Until March 1996 all blood samples were sent to the National Institute of Forensic Toxicology (NIFT, for analysis.NIFT routinely perform only those analyses requested, but the samples are stored for up to two years afteranalysis. By later additional analyses of this material one could obtain a more

  16. Hvor moralsk tenker fotballspillere? – en empirisk studie av toppfotball

    Directory of Open Access Journals (Sweden)

    Lars Tore Ødegård

    2015-11-01

    Full Text Available Det er en utbredt oppfatning at det økende prestasjonskravet i toppfotball fører til en svekkelse av moral og fair play-holdninger. Men hvordan tenker toppfotballspillere i forhold til ulike dilemmaer som oppstår på banen, og er det forskjeller mellom toppfotballspillere og spillere på lavere nivå?  For å avklare dette gjennomførte vi en empirisk undersøkelse der vi intervjuet spillere og trener i én toppklubb og én breddeklubb. Vi var interessert i to hovedproblemstillinger:  1 Hva tenker fotballspillere på ulike prestasjonsnivå om etikk og moralske dilemmaer i fotball? 2 Hvilke faktorer påvirker spillerne, direkte og indirekte, i deres vurderinger og handlinger? Resultatene fra undersøkelsen viser at det er store forskjeller mellom de to prestasjonsnivåene. Spillerne på breddenivået føler generelt et større ansvar for å opptre ærlig på banen enn hva som er tilfelle på toppnivået. Vi fant 4 spesifikke faktorer i toppfotballen som bidrar til umoralsk opptreden. For det første opplever spillerne en forventning om at de prioriterer resultat foran fair play. For det andre bidrar tilstedeværelsen av supportere på kamper til en slik prioritering. For det tredje vil handlinger som er i strid med fair play signaliseres som akseptable internt i miljøet, selv om de ofte signaliseres som uakseptable overfor media og omverdenen. For det fjerde kan spillere oppleve det som vanskelig å gå i mot trenerens oppfordringer til en pragmatisk og resultatfokusert opptreden.Nøkkelord: moral, etikk, fair play, footballEnglish summary: How morally do football players think? – an empirical study of moral dilemmas in elite footballIt is a common view that the increasing focus on achievement and success in elite football leads to a weakening of moral and fair play attitudes. But how do elite players think and what do they think about different dilemmas that appear on the field, and is there a difference between elite players and lower

  17. Vurdering av andrespråksinnlærere - en utfordring i skolen

    Directory of Open Access Journals (Sweden)

    Kirsten Palm

    2014-06-01

    Full Text Available Vurdering av andrespråkselevers språkkompetanse og språkutvikling er en sentral del av læreres arbeid, og forskning har vist at dette er en utfordring for skolene. Det er stor variasjon i vurderingsformer, og skolene mangler ofte vurderingskompetanse. På bakgrunn av dette utarbeidet Utdanningsdirektoratet i 2007 vurderingsverktøyet Kartleggingsmateriell. Språkkompetanse i grunnleggende norsk for språklige minoriteter. Materiellet er utarbeidet blant annet med referanse til Common European Framework of Reference for Languages (CEFR. I artikkelen redegjør vi for en undersøkelse der målet var å finne ut hvorvidt dette kartleggingsmateriellet er hensiktsmessig for å vurdere andrespråkselevers språkkompetanse. Hoveddataene er innhentet gjennom kasusstudier ved tre grunnskoler, der vi intervjuet skoleledelse og lærere samt observerte kartleggingssituasjoner og klasseromsundervisning. Kasusstudiene ble senere fulgt opp av to spørreundersøkelser, én til de samme skolene, og én til et større antall skoler for å få bredere innsikt i kartleggingspraksis og skolers oppfatninger av denne formen for vurdering. Undersøkelsen viser at flere av informantene mener verktøyet kan bidra til mer tilpasset og systematisk andrespråkopplæring og bedre vurdering av andrespråkselevenes språkkompetanse. Samtidig er det utfordringer knyttet til å bruke vurderingsverktøyet, dette skyldes blant annet mangelfull kompetanse i skolen når det gjelder andrespråkstilegnelse og vurdering av elevenes språkkompetanse. Det er ikke tidligere forsket på bruk av et slikt konkret kartleggingsverktøy i norsk grunnskole. Undersøkelsen kan gi et bidrag til økt kunnskap om vurdering av andrespråkskompetanse og tilpasset undervisning for andrespråksinnlærere.

  18. Sampling method, storage and pretreatment of sediment affect AVS concentrations with consequences for bioassay responses

    Energy Technology Data Exchange (ETDEWEB)

    Lange, H.J. de [Aquatic Ecology and Water Quality Management Group, Wageningen University, Wageningen University and Research Centre, P.O. Box 8080, 6700 DD, Wageningen (Netherlands); Centre for Ecosystem Studies, Alterra, Wageningen University and Research Centre, P.O. Box 47, 6700 AA, Wageningen (Netherlands)], E-mail: marieke.delange@wur.nl; Griethuysen, C. van; Koelmans, A.A. [Aquatic Ecology and Water Quality Management Group, Wageningen University, Wageningen University and Research Centre, P.O. Box 8080, 6700 DD, Wageningen (Netherlands)

    2008-01-15

    Sediment treatment and sediment storage may alter sediment toxicity, and consequently biotic response. Purpose of our study was to combine these three aspects (treatment-toxicity-biotic response) in one integrated approach. We used Acid Volatile Sulfide (AVS) concentrations as a proxy of the disturbance of the sediment. AVS and Simultaneously Extracted Metal (SEM) concentrations were compared to bioassay responses with the freshwater benthic macroinvertebrate Asellus aquaticus. Storage conditions and sediment treatment affected AVS but not SEM levels. AVS can be used as a proxy for sediment disturbance. The best way to pretreat the sediment for use in a bioassay in order to maintain initial AVS conditions was to sample the sediment with an Ekman grab, immediately store it in a jar without headspace, and freeze it as soon as possible. In a survey using seven different sediments, bioassay responses of A. aquaticus were correlated with SEM and AVS characteristics. - Change in AVS is a good proxy for sediment disturbance and combined with SEM it can be used as a suitable predictor for biotic effects of sediment contamination.

  19. Bruk av rusmidler blant norske 15-16 åringer. Resultater fra den norske delen av de europeiske skoleundersøkelsene - ESPAD 1995, 1999 og 2003

    OpenAIRE

    Bye, Elin Kristin; Skretting, Astrid

    2003-01-01

    - Tobakk Omkring 60 prosent av 15 - 16 åringene oppga i 2003 at de noen gang har røykt tobakk, omkring 30 prosent hadde røykt i løpet av de siste 30 dager. En andel på 18 prosent hadde røykt daglig i løpet av siste måned. Det er flere jenter enn gutter som oppgir at de røyker. Mens det var en økning fra 1995 til 1999 i andelen 15 - 16 åringer som røyker tobakk, var det en nedgang fra 1999 til 2003. En andel på fire prosent oppga i 2003 at de hadde røykt ...

  20. Helsekonsekvenser av vektendring - er slanking fordelaktig? En litteraturoversikt

    Directory of Open Access Journals (Sweden)

    Anne Johanne Søgaard

    2009-11-01

    Full Text Available  SAMMENDRAGLangsom vektøkning gjennom livet inntil kroppsmasseindeks (KMI 27 ser ikke ut til å gi særlige negative utfallmht. total dødelighet. En økning på ! 20 kg fra 18 års alder er trolig negativt – det samme er vektøkning ut fra enhøy utgangsvekt. Selv om mange studier finner økt dødelighet hos yngre og middelaldrende personer med KMI > 27,er det foreløpig ingen studier som har vist at vektreduksjon blant friske overvektige personer gir økt levetid. Flerestudier viser tvert imot en assosiasjon mellom vektreduksjon og økt dødelighet. Heller ikke studier som prøver åbegrense analysene til personer med frivillig vekttap, finner redusert dødelighet etter vektreduksjon blant friskeovervektige individer. Noen få studier har vist redusert dødelighet blant overvektige personer med etablertesykdommer eller høy risiko for hjerte-karsykdom og diabetes. Når det gjelder sykelighet har de fleste studier entenikke funnet noen assosiasjon mellom vektreduksjon og sykelighet, eller funnet økt sykelighet. Unntaket er diabetes,der vekttap i enkelte studier samvarierer med redusert sykelighet. Det er i mange studier funnet sammenheng mellomvekttap og økt risiko for lårhalsbrudd og gallesten. De fleste studier har imidlertid vist at moderat vektreduksjon(vekttap på opp til 10% fører til redusert blodtrykk, forbedret glukosekontroll, redusert kolesterolnivå, forbedretdyslipidemi, forbedret lungefunksjon, bedre søvnkvalitet og redusert søvnapnè. Moderat vekttap vil også kunnelindre leddsmerter. Dette tilsynelatende paradoks diskuteres i artikkelen – bl.a. drøftes de metodeproblemer som erinnebygget i mange av studiene. Fordi det har vist seg vanskelig for personer med kraftig overvekt å redusere vektenpermanent – og fordi resultatene mht. helseeffektene av vektreduksjon er usikre, bør hovedinnsatsen settes inn iforhold til å forebygge overvekt/fedme overfor enkeltpersoner med potensielle problemer. Personer med