Results 91 to 100 of about 29,744 (185)

Progressive multifocal diffusion-weighted imaging hyperintensities in sporadic Creutzfeldt–Jakob disease with positive cerebrospinal fluid real-time quaking-induced conversion: a case report

Journal of Medical Case Reports
Background Creutzfeldt–Jakob disease is a rare and invariably fatal neurodegenerative disorder classified among the prion diseases. Primarily affecting the brain, this condition precipitates neuronal cell demise, culminating in swiftly advancing dementia
Puyu Li, Wenjing Wang, Lingzhi Li, Keding Cheng, Ying Wang, Yuanyuan Li, Wei Xu   +6 more
doaj   +1 more source

The first reported case of Creutzfeldt‐Jakob disease from Nepal

Clinical Case Reports, 2020
Creutzfeldt‐Jakob disease (CJD) can also be diagnosed in a resource‐limited setting through good clinical analysis. The diagnosis of CJD should be considered in patients with rapidly evolving neurological signs associated with cognitive disturbances even
Himal Kharel, Pabitra Adhikari, Nishan B. Pokhrel, Zeni Kharel, Gaurav Nepal   +4 more
doaj   +1 more source

Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease [PDF]

, 2006
To validate the provisional findings of a number of smaller studies and explore additional determinants of characteristic diagnostic investigation results across the entire clinical spectrum of sporadic Creutzfeldt-Jakob disease (CJD), an international ...
Almonti, S. (S.), Alperovitch, A. (Annick), Brandel, J-P. (Jean-Philippe), Budka, H. (Herbert), Collins, S.J. (Steven), Cuadrado-Corrales, N. (Natividad), Duijn, C.M. (Cornelia) van, Gelpi, E. (Ellen), Glatzel, M. (Markus), Heinemann, U., Hewer, E., Jansen, G.H. (Gerard), Klug, G.M. (Genevieve), Kretszchmar, H.A. (Hans), Mackenzie, J., Masters, C. (Colin), Mitrova, E. (E.), Murray, K. (K.), Olsen, E. (E.), Pedro-Cuesta, J. (Jesús) de, Pocchiari, M. (Maurizio), Poleggi, A. (Anna), Sanchez-Juan, P. (Pascual), Tolnay, M. (Markus), Will, R.G. (Robert), Zerr, I. (Inga)   +25 more
core   +1 more source

Demyelinating neuropathy as the initial presentation of familial E200K Creutzfeldt–Jakob disease in two patients

Annals of Clinical and Translational Neurology
Objective To describe peripheral neuropathy associated with familial Creutzfeldt‐Jakob disease. Methods We report two unrelated patients with genetic Creutzfeldt–Jakob disease with demyelinating peripheral neuropathy as initial presentation, with a ...
Cécile Delorme, Antoine Pégat, Julian Theuriet, Jean‐Philippe Brandel, Emmanuel Roze, Karine Viala, Julie Zyss, Stéphane Thobois, Anthony Fourier, Emilien Bernard, Juliette Svahn, Chloé Laurencin, Paul Jaulent, Christophe Vandendries, Isabelle Quadrio, Virginie Desestret, David Meyronet, Thierry Maisonobe, Stéphane Haïk, Danielle Seilhean   +19 more
doaj   +1 more source

Shortest known prion protein allele in highly BSE-susceptible lemurs [PDF]

, 2000
We describe the shortest prion protein allele known to date. Surprisingly, it is found as a polymorphism exactly in a species (prosimian lemurs) which seems highly susceptible to oral infection with BSE-derived prions. The truncation of the prion protein
Gilch, S., Schätzl, H. M., Spielhaupter, C.   +2 more
core   +1 more source

Rapidly progressive dementia: probable sporadic Creutzfeldt–Jakob disease in a Yoruba Nigerian woman with rapidly progressive dementia: a case report

Journal of Medical Case Reports
Background Creutzfeldt–Jakob disease is a neurodegenerative disorder that can present with neuropsychiatric features such as dementia; it is a rare cause of rapidly progressive dementia.
A. O. Idowu, A. F. Ogunmodede, A. A. Sanusi, U. C. Eke, S. A. Oyedele, M. B. Fawale, M. A. Komolafe   +6 more
doaj   +1 more source

Assignment of the Human and Mouse Prion Protein Genes to Homologous Chromosomes [PDF]

, 1986
Purified preparations of scrapie prions contain one major macromolecule, designated prion protein (PrP). Genes encoding PrP are found in normal animals and humans but not within the infectious particles.
Blatt, Cila, Cohn, Vivian H., DeArmond, Stephen J., Fournier, R. E. K., Heinzmann, Camilla, Klisak, Ivana, Lem, Janice, Lucero, Michael, Mohandas, T., Pruisner, Stanley B., Simon, Melvin, Sparkes, Robert S., Weiner, Leslie P., Westaway, David   +13 more
core  

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

PLoS Pathogens, 2006
Transmission of prions between species is limited by the "species barrier," which hampers a full characterization of human prion strains in the mouse model.
Romolo Nonno, Michele A Di Bari, Franco Cardone, Gabriele Vaccari, Paola Fazzi, Giacomo Dell'Omo, Claudia Cartoni, Loredana Ingrosso, Aileen Boyle, Roberta Galeno, Marco Sbriccoli, Hans-Peter Lipp, Moira Bruce, Maurizio Pocchiari, Umberto Agrimi   +14 more
doaj   +2 more sources

Wariant Choroby Creutzfeldta-Jakoba: aktualny stan wiedzy

Aktualności Neurologiczne, 2011
Choroby wywoływane przez priony obejmują: chorobę Creutzfeldta-Jakoba (Creutzfeldt-Jakob disease, CJD), kuru, chorobę Gerstmanna-Sträusslera-Scheinkera (GSS), śmiertelną rodzinną bezsenność (fatal familial insomnia, FFI) u człowieka, scrapie (polska ...
James W. Ironside
doaj  

Constant Transmission Properties of Variant Creutzfeldt-Jakob Disease in 5 Countries [PDF]

, 2012
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.
Abigail B. Diack, Bird, Brandel, Bruce, Bruce, Bruce, Chadeau-Hyam, Cornelia Van Duijn, Diane Ritchie, Dickinson, Ermias D. Belay, Fabrizio Tagliavini, Fraser, Head, Hill, Jean C. Manson, Jean-Philippe Brandel, Lawrence B. Schonberger, Matthew Bishop, Pedro Piccardo, Pierluigi Gambetti, Ritchie, Robert G. Will, Sanchez-Juan, Stephane Haik, Victoria Pinion, Will   +26 more
core   +5 more sources