Results 41 to 50 of about 29,456 (167)

The human spongiform encephalopathies [PDF]

Romanian Journal of Neurology, 2018
The human spongiform encephalopathies are a group of heterogenous, usually fatal diseases, characterized by a unique pathogenetic mechanism and distinct clinical presentation. They are classified into sporadic, familial and acquired forms.
Mavroudis Ioannis, Petrides Foivos, Kazis Dimitrios   +2 more
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Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease [PDF]

, 2017
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.
Begue, C., Brandel, J. P., Breithaupt, M., Collie, D., Collins, S. J., Cras, P., Haik, S., Heinemann, U., Hewer, E., Jansen, Gerard H., Kallenberg, K., Ladogana, A., Meissner, B., Pimentel, J., Pocchiari, M., Roberts, H., Romero, C., Sanchez-Juan, P., Schuur, M., Smith, P., Stokin, G. B., Summers, D. M., Taratuto, A., van Duijn, C., Varges, D., Will, R. G., Zerr, I.   +26 more
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Cerebrospinal fluid biomarker supported diagnosis of Creutzfeldt-Jakob disease and rapid dementias: a longitudinal multicentre study over 10 years [PDF]

, 2017
To date, cerebrospinal fluid analysis, particularly protein 14-3-3 testing, presents an important approach in the identification of Creutzfeldt-Jakob disease cases.
Aguzzi, Adriano, Calero, Miguel, Gawinecka, Joanna, Green, Alison, Karch, Andre, Knight, Richard, Kulczycki, Jerzy, Ladogana, Anna, Laplanche, Jean-Louis, Mitrova, Eva, Peoc'h, Katell, Pocchiari, Maurizio, Saiz, Albert, Sanchez-Juan, Pascual, Sanchez-Valle, Raquel, Schelzke, Gabi, Sklaviadis, Theodor, Slivarichova, Dana, Stoeck, Katharina, van Duijn, Cornelia M., Zerr, Inga   +20 more
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Human stem cell-derived astrocytes replicate human prions in a PRNP genotype-dependent manner. [PDF]

, 2017
Prions are infectious agents that cause neurodegenerative diseases such as Creutzfeldt-Jakob disease (CJD). The absence of a human cell culture model that replicates human prions has hampered prion disease research for decades.
Alibhai, James, Chandran, Siddharthan, Head, Mark W, Ironside, James W, Krejciova, Zuzana, Krencik, Robert, Manson, Jean, Rzechorzek, Nina M, Ullian, Erik M, Zhao, Chen   +9 more
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THE CLINICAL CASE OF PRIMARY PROGRESSIVE APHASIA

Учёные записки Санкт-Петербургского государственного медицинского университета им. Акад. И.П. Павлова, 2019
The article described the rare clinical case of the patient with probable transmissible encephalopathy (Creutzfeldt–Jakob disease) in whom rapidly forming primary progressive aphasia was the herald clinical feature, and motor and sensory functions were ...
Natalya V. Shuleshova, Aleksey V. Sizov, Irina V. Kupriianova, Victoria V. Zvereva   +3 more
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The Molecular Pathology of Prion Diseases [PDF]

, 2004
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal ...
Herms, Jochen, Kretzschmar, Hans A., Vassallo, Neville   +2 more
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Gerstmann-Sträussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments [PDF]

, 2019
Despite their phenotypic heterogeneity, most human prion diseases belong to two broadly defined groups: Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker disease (GSS).
Cali, Ignazio, Cracco, Laura, Gambetti, Pierluigi, Ghetti, Bernardino, Lavrich, Jody, Nemani, Satish K., Notari, Silvio, Surewicz, Witold K., Xiao, Xiangzhu   +8 more
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Applicability of long-term electroencephalography in pre-mortem diagnosis of Creutzfeldt–Jakob disease: A case report

SAGE Open Medical Case Reports, 2017
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
Sanaz Attaripour Isfahani, Michelle Dougherty, Gediminas Peter Gliebus   +2 more
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Different forms of the bovine PrP gene have five or six copies of a short, G-C-rich element within the protein-coding exon [PDF]

, 1991
Current models of the virus-like agents of scrapie and bovine spongiform encephalopathy (BSE) have to take into account that structural changes in a host-encoded protein (PrP protein) exhibit an effect on the time course of these diseases and the ...
Dawson, M, Goldmann, W, Hope, J, Hunter, N, Martin, T   +4 more
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High diagnostic performance of plasma and cerebrospinal fluid beta‐synuclein for sporadic Creutzfeldt–Jakob disease

Annals of Clinical and Translational Neurology, 2023
Beta‐synuclein is a promising cerebrospinal fluid and blood biomarker of synaptic damage. Here we analysed its accuracy in the discrimination between sporadic Creutzfeldt–Jakob disease (n = 150) and non‐prion rapidly progressive dementias (n = 106).
Samir Abu‐Rumeileh, Steffen Halbgebauer, Giuseppe Mario Bentivenga, Lorenzo Barba, Simone Baiardi, Andrea Mastrangelo, Patrick Oeckl, Petra Steinacker, Angela Mammana, Sabina Capellari, Markus Otto, Piero Parchi   +11 more
doaj   +1 more source