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[PMID]:28449707
[Au] Autor:Llorens F; Thüne K; Sikorska B; Schmitz M; Tahir W; Fernández-Borges N; Cramm M; Gotzmann N; Carmona M; Streichenberger N; Michel U; Zafar S; Schuetz AL; Rajput A; Andréoletti O; Bonn S; Fischer A; Liberski PP; Torres JM; Ferrer I; Zerr I
[Ad] Endereço:Department of Neurology, University Medical Center Göttingen, and German Center for Neurodegenerative Diseases (DZNE), Robert Koch Strasse 40, 37075, Göttingen, Germany. franc.llorens@gmail.com.
[Ti] Título:Altered Ca homeostasis induces Calpain-Cathepsin axis activation in sporadic Creutzfeldt-Jakob disease.
[So] Source:Acta Neuropathol Commun;5(1):35, 2017 04 27.
[Is] ISSN:2051-5960
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo: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 ). The molecular mechanisms underlying these alterations are largely unknown, but the presence of intracellular neuronal calcium (Ca ) 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 responsive genes in sCJD brain tissue, accompanied by two Ca -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 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 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.
[Mh] Termos MeSH primário: Encéfalo/metabolismo
Cálcio/metabolismo
Calpaína/metabolismo
Catepsinas/metabolismo
Síndrome de Creutzfeldt-Jakob/metabolismo
Homeostase/fisiologia
[Mh] Termos MeSH secundário: Animais
Encéfalo/patologia
Cátions Bivalentes/metabolismo
Células Cultivadas
Síndrome de Creutzfeldt-Jakob/patologia
Modelos Animais de Doenças
Seres Humanos
Lisossomos/metabolismo
Lisossomos/patologia
Mesocricetus
Camundongos Transgênicos
Neurônios/metabolismo
Neurônios/patologia
Proteínas PrPSc/metabolismo
Ratos Wistar
Proteínas Recombinantes/metabolismo
Ovinos
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Cations, Divalent); 0 (PrPSc Proteins); 0 (Recombinant Proteins); EC 3.4.- (Cathepsins); EC 3.4.22.- (Calpain); SY7Q814VUP (Calcium)
[Em] Mês de entrada:1712
[Cu] Atualização por classe:180307
[Lr] Data última revisão:
180307
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170429
[St] Status:MEDLINE
[do] DOI:10.1186/s40478-017-0431-y


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[PMID]:29365304
[Au] Autor:Narula R; Tinaz S
[Ad] Endereço:Yale New Haven Hospital, New Haven, CT reshma.narula@yale.edu.
[Ti] Título:Creutzfeldt-Jakob Disease.
[So] Source:N Engl J Med;378(4):e7, 2018 Jan 25.
[Is] ISSN:1533-4406
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Encéfalo/diagnóstico por imagem
Síndrome de Creutzfeldt-Jakob/diagnóstico
[Mh] Termos MeSH secundário: Proteínas 14-3-3/líquido cefalorraquidiano
Encéfalo/patologia
Transtornos Cognitivos/etiologia
Síndrome de Creutzfeldt-Jakob/complicações
Síndrome de Creutzfeldt-Jakob/diagnóstico por imagem
Eletroencefalografia
Evolução Fatal
Seres Humanos
Imagem por Ressonância Magnética
Masculino
Meia-Idade
Proteínas tau/líquido cefalorraquidiano
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (14-3-3 Proteins); 0 (tau Proteins)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180208
[Lr] Data última revisão:
180208
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180125
[St] Status:MEDLINE
[do] DOI:10.1056/NEJMicm1710121


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[PMID]:29310343
[Au] Autor:Yao Y; Dong X; Guan H; Lu Q
[Ad] Endereço:Department of Neurology, Peking Union Medical College Hospital, Dongcheng.
[Ti] Título:Cerebrospinal fluid real-time quaking-induced conversion test for sporadic Creutzfeldt-Jakob disease in an 18-year-old woman: A case report.
[So] Source:Medicine (Baltimore);96(48):e8699, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Sporadic Creutzfeldt-Jakob disease (sCJD) mainly occurs in the elderly, with the peak age of onset ranging from 55 to 75 years. The symptoms of sCJD are not unique, and laboratory tests such as magnetic resonance imaging (MRI), electroencephalogram (EEG) and cerebrospinal fluid (CSF)14-3-3 protein have low sensitivity or specificity. Therefore, excluding treatable diseases and establishing a diagnosis could be difficult in young patients with suspected sCJD. Recently, real-time quaking-induced conversion (RT-QuIC) has been used in the diagnosis of sCJD, with more than 95% sensitivity and 100% specificity. PATIENT CONCERNS: We report the case of an 18-year-old woman presented with cerebellar ataxia, blurred vision, rapidly progressive dementia, tremor and involuntary movements, urinary incontinence, mutism, and eventually myoclonus for 16 weeks. Brain MRI scans were unremarkable at the 4th and 8th week after initial symptom presentation, but showed hyperintensity in bilateral basal ganglia and cortical ribboning at the 16th week. Typical periodic bilateral triphasic sharp wave complexes on EEG did not appear until the 16th week after initial symptom presentation. DIAGNOSES: Due to the young age of the patient and the originally unremarkable MRI and EEG findings, we first considered treatable diseases such as autoimmune encephalitis, infections, organic acidemias and toxication. However, extensive tests ruled out these diseases. When she was finally diagnosed with probable sCJD, we were unable to perform a brain biopsy. We confirmed the diagnosis by detecting the scrapie form of prion protein in the CSF using RT-QuIC. INTERVENTIONS: Experimental treatments with corticosteroids, intravenous immunoglobulin and ganciclovir were given. OUTCOMES: Experimental treatments were ineffective. The patient's parents discharged her from our clinic. LESSONS: We present a case of probable sCJD with an early onset and a complex clinical picture confirmed by RT-QuIC. This case report suggests that RT-QuIC has great value for the diagnosis of atypical cases.
[Mh] Termos MeSH primário: Síndrome de Creutzfeldt-Jakob/líquido cefalorraquidiano
[Mh] Termos MeSH secundário: Adolescente
Diagnóstico Diferencial
Eletroencefalografia
Feminino
Seres Humanos
Imagem por Ressonância Magnética
Sensibilidade e Especificidade
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180115
[Lr] Data última revisão:
180115
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180110
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000008699


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[PMID]:29190276
[Au] Autor:Diack AB; Will RG; Manson JC
[Ad] Endereço:The Roslin Institute & R(D)SVS, University of Edinburgh, Easter Bush, United Kingdom.
[Ti] Título:Public health risks from subclinical variant CJD.
[So] Source:PLoS Pathog;13(11):e1006642, 2017 11.
[Is] ISSN:1553-7374
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Síndrome de Creutzfeldt-Jakob
[Mh] Termos MeSH secundário: Animais
Síndrome de Creutzfeldt-Jakob/diagnóstico
Síndrome de Creutzfeldt-Jakob/transmissão
Seres Humanos
Saúde Pública
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T; REVIEW
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180102
[Lr] Data última revisão:
180102
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171201
[St] Status:MEDLINE
[do] DOI:10.1371/journal.ppat.1006642


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[PMID]:28924012
[Au] Autor:Bertani I; Iori V; Trusel M; Maroso M; Foray C; Mantovani S; Tonini R; Vezzani A; Chiesa R
[Ad] Endereço:Department of Neuroscience, Istituto di Ricovero e Cura a Carattere Scientifico-Istituto di Ricerche Farmacologiche Mario Negri, 20156 Milan, Italy, and.
[Ti] Título:Inhibition of IL-1ß Signaling Normalizes NMDA-Dependent Neurotransmission and Reduces Seizure Susceptibility in a Mouse Model of Creutzfeldt-Jakob Disease.
[So] Source:J Neurosci;37(43):10278-10289, 2017 Oct 25.
[Is] ISSN:1529-2401
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo: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-1ß-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-1ß plays a role in the enhanced synaptic responsiveness and seizure susceptibility, suggesting that targeting IL-1ß signaling may offer a novel symptomatic treatment for CJD. 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 brain levels of the inflammatory cytokine IL-1ß. Here we show that blocking IL-1ß receptors with anakinra, the human recombinant form of the endogenous IL-1 receptor antagonist used to treat rheumatoid arthritis, normalizes hippocampal neurotransmission and reduces seizure susceptibility in a CJD mouse model. These results link neuroinflammation to defective neurotransmission and the enhanced susceptibility to seizures in CJD and raise the possibility that targeting IL-1ß with clinically available drugs may be beneficial for symptomatic treatment of the disease.
[Mh] Termos MeSH primário: Síndrome de Creutzfeldt-Jakob/tratamento farmacológico
Modelos Animais de Doenças
Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico
Interleucina-1beta/antagonistas & inibidores
Receptores de N-Metil-D-Aspartato/antagonistas & inibidores
Convulsões/tratamento farmacológico
[Mh] Termos MeSH secundário: Animais
Síndrome de Creutzfeldt-Jakob/metabolismo
Suscetibilidade a Doenças
Feminino
Hipocampo/efeitos dos fármacos
Hipocampo/metabolismo
Seres Humanos
Proteína Antagonista do Receptor de Interleucina 1/farmacologia
Interleucina-1beta/metabolismo
Masculino
Camundongos
Camundongos Endogâmicos C57BL
Camundongos Knockout
Camundongos Transgênicos
N-Metilaspartato/antagonistas & inibidores
N-Metilaspartato/metabolismo
Plasticidade Neuronal/efeitos dos fármacos
Plasticidade Neuronal/fisiologia
Distribuição Aleatória
Receptores de N-Metil-D-Aspartato/metabolismo
Convulsões/metabolismo
Transdução de Sinais/efeitos dos fármacos
Transdução de Sinais/fisiologia
Transmissão Sináptica/efeitos dos fármacos
Transmissão Sináptica/fisiologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Interleukin 1 Receptor Antagonist Protein); 0 (Interleukin-1beta); 0 (Receptors, N-Methyl-D-Aspartate); 6384-92-5 (N-Methylaspartate)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171106
[Lr] Data última revisão:
171106
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170920
[St] Status:MEDLINE
[do] DOI:10.1523/JNEUROSCI.1301-17.2017


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[PMID]:28821618
[Au] Autor:Privat N; Levavasseur E; Yildirim S; Hannaoui S; Brandel JP; Laplanche JL; Béringue V; Seilhean D; Haïk S
[Ad] Endereço:From the INSERM, UMR S1127, 75013 Paris, France.
[Ti] Título:Region-specific protein misfolding cyclic amplification reproduces brain tropism of prion strains.
[So] Source:J Biol Chem;292(40):16688-16696, 2017 Oct 06.
[Is] ISSN:1083-351X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Human prion diseases such as Creutzfeldt-Jakob disease are transmissible brain proteinopathies, characterized by the accumulation of a misfolded isoform of the host cellular prion protein (PrP) in the brain. According to the prion model, prions are defined as proteinaceous infectious particles composed solely of this abnormal isoform of PrP (PrP ). Even in the absence of genetic material, various prion strains can be propagated in experimental models. They can be distinguished by the pattern of disease they produce and especially by the localization of PrP deposits within the brain and the spongiform lesions they induce. The mechanisms involved in this strain-specific targeting of distinct brain regions still are a fundamental, unresolved question in prion research. To address this question, we exploited a prion conversion assay, protein misfolding cyclic amplification (PMCA), by using experimental scrapie and human prion strains as seeds and specific brain regions from mice and humans as substrates. We show here that region-specific PMCA in part reproduces the specific brain targeting observed in experimental, acquired, and sporadic Creutzfeldt-Jakob diseases. Furthermore, we provide evidence that, in addition to cellular prion protein, other region- and species-specific molecular factors influence the strain-dependent prion conversion process. This important step toward understanding prion strain propagation in the human brain may impact research on the molecular factors involved in protein misfolding and the development of ultrasensitive methods for diagnosing prion disease.
[Mh] Termos MeSH primário: Encéfalo/metabolismo
Síndrome de Creutzfeldt-Jakob/metabolismo
Proteínas PrPSc/metabolismo
Dobramento de Proteína
Deficiências na Proteostase/metabolismo
[Mh] Termos MeSH secundário: Animais
Encéfalo/patologia
Síndrome de Creutzfeldt-Jakob/genética
Síndrome de Creutzfeldt-Jakob/patologia
Seres Humanos
Camundongos
Camundongos Transgênicos
Proteínas PrPSc/genética
Isoformas de Proteínas/genética
Isoformas de Proteínas/metabolismo
Deficiências na Proteostase/genética
Deficiências na Proteostase/patologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (PrPSc Proteins); 0 (Protein Isoforms)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171011
[Lr] Data última revisão:
171011
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170820
[St] Status:MEDLINE
[do] DOI:10.1074/jbc.M117.793646


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[PMID]:28791720
[Au] Autor:Huor A; Douet JY; Lacroux C; Lugan S; Tillier C; Aron N; Cassard H; Arnold M; Torres JM; Ironside JW; Andréoletti O
[Ad] Endereço:UMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Vétérinaire de Toulouse, France.
[Ti] Título:Infectivity in bone marrow from sporadic CJD patients.
[So] Source:J Pathol;243(3):273-278, 2017 Nov.
[Is] ISSN:1096-9896
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Prion infectivity was recently identified in the blood of both sporadic and variant Creutzfeldt-Jakob disease (CJD) patients. In variant CJD (vCJD), the widespread distribution of prions in peripheral tissues of both asymptomatic and symptomatic patients is likely to explain the occurrence of the observed prionaemia. However, in sporadic CJD (sCJD), prion infectivity is described to be located principally in the central nervous system. In this study, we investigated the presence of prion infectivity in bone marrow collected after death in patients affected with different sCJD agents. Bioassays in transgenic mice expressing the human prion protein revealed the presence of unexpectedly high levels of infectivity in the bone marrow from seven out of eight sCJD cases. These findings may explain the presence of blood-borne infectivity in sCJD patients. They also suggest that the distribution of prion infectivity in peripheral tissues in sCJD patients could be wider than currently believed, with potential implications for the iatrogenic transmission risk of this disease. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
[Mh] Termos MeSH primário: Medula Óssea/metabolismo
Síndrome de Creutzfeldt-Jakob/metabolismo
Proteínas Priônicas/metabolismo
[Mh] Termos MeSH secundário: Idoso
Animais
Síndrome de Creutzfeldt-Jakob/genética
Síndrome de Creutzfeldt-Jakob/patologia
Modelos Animais de Doenças
Feminino
Seres Humanos
Masculino
Camundongos Transgênicos
Meia-Idade
Príons/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Prion Proteins); 0 (Prions)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171107
[Lr] Data última revisão:
171107
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170810
[St] Status:MEDLINE
[do] DOI:10.1002/path.4954


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[PMID]:28668775
[Au] Autor:Baiardi S; Magherini A; Capellari S; Redaelli V; Ladogana A; Rossi M; Tagliavini F; Pocchiari M; Giaccone G; Parchi P
[Ad] Endereço:Università di Bologna, Dipartimento di Scienze Biomediche e Neuromotorie, Bologna, Italy.
[Ti] Título:Towards an early clinical diagnosis of sporadic CJD VV2 (ataxic type).
[So] Source:J Neurol Neurosurg Psychiatry;88(9):764-772, 2017 Sep.
[Is] ISSN:1468-330X
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: Sporadic Creutzfeldt-Jakob disease (sCJD) includes a broad spectrum of clinical-pathological subtypes, which complicates the clinical differential diagnosis with other rapidly progressive neurological syndromes. AIM: To provide a better characterisation of clinical features and results of diagnostic investigations, especially at an early disease stage, in patients with sCJDVV2, the second most common sCJD subtype. METHODS: We evaluated neurological symptoms/signs, and results of brain diffusion-weighted resonance imaging (DW-MRI), electroencephalographic recordings (EEG) and cerebrospinal fluid (CSF) biomarker studies in 120 patients with a definite (n=93) or probable (n=27) diagnosis of sCJDVV2. RESULTS: All patients presented with prominent cerebellar signs, which were often associated with memory loss and/or oculomotor, visual or peripheral/spinal cord signs. In contrast, dementia was invariably a late finding. All CSF samples were positive for the 14-3-3 protein assay and had total-tau protein levels above 1250 pg/mL. Brain DW-MRI showed hyperintensity of basal ganglia, thalamus and cerebral cortex, respectively in 91.5%, 57.4% and 19.1% of cases. EEG revealed periodic sharp-wave complexes in only 17.8% of cases. CONCLUSIONS: sCJDVV2 should be considered in any patient presenting with a rapidly progressive ataxia, especially when associated with oculomotor, visual or peripheral/spinal cord signs, even in the absence of dementia or myoclonus. CSF assays and brain DW-MRI represent sensitive diagnostic tests, even at an early stage. These data strongly suggest that sCJDVV2 can be clinically diagnosed early and accurately based on clinical data, DW-MRI, CSF assays and codon 129 genotyping and provide the basis for improved and subtype-specific diagnostic criteria of sCJD.
[Mh] Termos MeSH primário: Ataxia/diagnóstico
Síndrome de Creutzfeldt-Jakob/diagnóstico
Diagnóstico Diferencial
Diagnóstico Precoce
[Mh] Termos MeSH secundário: Biomarcadores/líquido cefalorraquidiano
Encéfalo/patologia
Síndrome de Creutzfeldt-Jakob/classificação
Síndrome de Creutzfeldt-Jakob/genética
Imagem de Difusão por Ressonância Magnética/métodos
Eletroencefalografia
Feminino
Seres Humanos
Masculino
Meia-Idade
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:171114
[Lr] Data última revisão:
171114
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170703
[St] Status:MEDLINE
[do] DOI:10.1136/jnnp-2017-315942


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[PMID]:28444687
[Au] Autor:Crowder LA; Schonberger LB; Dodd RY; Steele WR
[Ad] Endereço:Scientific Affairs, American Red Cross, Rockville, Maryland.
[Ti] Título:Creutzfeldt-Jakob disease lookback study: 21 years of surveillance for transfusion transmission risk.
[So] Source:Transfusion;57(8):1875-1878, 2017 Aug.
[Is] ISSN:1537-2995
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: 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. STUDY DESIGN AND METHODS: 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. RESULTS: 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. CONCLUSION: From this study, as well as other epidemiologic studies, there is no evidence of CJD transfusion transmission; this risk remains theoretical.
[Mh] Termos MeSH primário: Síndrome de Creutzfeldt-Jakob/etiologia
Monitoramento Epidemiológico
Doenças Priônicas/transmissão
Reação Transfusional
[Mh] Termos MeSH secundário: Doadores de Sangue
Inquéritos Epidemiológicos
Seres Humanos
Meia-Idade
Vigilância da População
Cruz Vermelha
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170427
[St] Status:MEDLINE
[do] DOI:10.1111/trf.14145


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[PMID]:28319876
[Au] Autor:Shin JW; Yim B; Oh SH; Kim NK; Lee SK; Kim OJ
[Ad] Endereço:Department of Neurology, CHA Bundang Medical Center, CHA University, Seongnam, South Korea.
[Ti] Título:Redefining periodic patterns on electroencephalograms of patients with sporadic Creutzfeldt-Jakob disease.
[So] Source:Clin Neurophysiol;128(5):756-762, 2017 May.
[Is] ISSN:1872-8952
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: 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. METHODS: We analyzed EEG data of 23 patients with sCJD were admitted to two university hospitals between August 2005 and September 2015. RESULTS: 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). CONCLUSIONS: 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. SIGNIFICANCE: 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.
[Mh] Termos MeSH primário: Síndrome de Creutzfeldt-Jakob/diagnóstico
Eletroencefalografia/normas
[Mh] Termos MeSH secundário: Idoso
Idoso de 80 Anos ou mais
Síndrome de Creutzfeldt-Jakob/fisiopatologia
Eletroencefalografia/métodos
Feminino
Seres Humanos
Masculino
Meia-Idade
Guias de Prática Clínica como Assunto/normas
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1705
[Cu] Atualização por classe:170530
[Lr] Data última revisão:
170530
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170321
[St] Status:MEDLINE



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