Results 21 to 30 of about 6,976 (171)

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

, 2009
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 ...
Maurizio Pocchiari, Roncaroli F, Cras, Patrick, Patrich Cras, Anna Ladogana, Giese, Armin, Cras, Patrich, Pocchiari M, Ladogana, Anna, Federico Roncaroli, Ladogana A, Bernardino Ghetti, Jan P. M. Langeveld, Strammiello, R., Zerr, I., Ghetti, B., Ghetti B, Roncaroli, Federico, Pocchiari, Maurizio, Langeveld, Jan P.M., Inga Zerr, Cras, P., Notari, Silvio, Cras P, Giese, A., Roncaroli, F., Parchi, Piero, Notari, S., Kretzschmar H, Langeveld JP, Rosaria Strammiello, Ghetti, Bernardino, Zerr, Inga, Kretzschmar, Hans A., Armin Giese, Parchi, P., Capellari, S., Hans Kretzschmar, Notari S, Pocchiari, M., Kretzschmar, Hans, Ladogana, A., Giese A, Langeveld, J.P.M., Capellari, Sabina, Strammiello, Rosaria, Piero Parchi, Silvio Notari, Zerr I, Langeveld, Jan P. M., Kretzschmar, H., Sabina Capellari   +51 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
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Sporadic MM-1 Type Creutzfeldt-Jakob Disease With Hemiballic Presentation and No Cognitive Impairment Until Death: How New NCJDRSU Diagnostic Criteria May Allow Early Diagnosis

Frontiers in Neurology, 2018
Sporadic Creutzfeldt-Jakob disease is the most common human prion disorder. Although associated with heterogeneous clinical phenotypes, its distinctive feature is the presence of a rapidly progressive multidomain cognitive impairment.
Lorenzo Saraceno, Vito A. G. Ricigliano, Michele Cavalli, Alessandro Cagol, Giovanna Bosco, Fabio Moda, Paola Caroppo, Giovanni Meola, Giovanni Meola   +8 more
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Beyond PrP res type 1/type 2 dichotomy in Creutzfeldt-Jakob disease [PDF]

, 2008
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 ...
Ironside, James W, Uro-Coste, Emmanuelle, Grassi, J., Head, Mark, Jean-Marc Bilheude, Lacroux, Caroline, Cassard Hervé, Armand Perret-Liaudet, Marie Bernadette Delisle, Grassi, Jacques, Head, M.W., Hauw, J.J., Head Mark W., Andréoletti Olivier, Bilheude, Jean-Marc, Peoch, Katell, Delisle, Marie-Bernadette, Streichenberger, Nathalie, Ironside, J.E., Simon Stéphanie, Lacroux Caroline, Simon, S., Haik, S., Peoch' Katell, Hervé Cassard, Jean-Jacques Hauw, Haik Stéphane, Basset-Leobon Christelle, Bilheude, J.M., Andreoletti, O., Jan Langeveld, Bilheude Jean-Marc, Stéphanie Simon, Uro-Coste Emmanuelle, Schelcher, F., Ironside, James, W., Langeveld, Jan, Christelle Basset-Leobon, Jacques Grassi, Olivier Andréoletti, Lugan, Séverine, Andréoletti, Olivier, Emmanuelle Uro-Coste, Lugan, S., Caroline Lacroux, Ironside, James, Hauw, Jean-Jacques, Perret-Liaudet, Armand, Basset-Leobon, Christelle, Basset-Leobon, C., Cassard, Hervé, Stéphane Haik, Head, Mark, W., Hauw Jean-Jacques, Langeveld Jan, James W Ironside, Simon, Stéphanie, Peoch, K., Séverine Lugan, Streichenberger Nathalie, Langeveld, J.P.M., Perret-Liaudet, A., Streichenberger, N., Cassard, H., Delisle, M.B., Uro-Coste, E., Haik, Stéphane, Schelcher, Francois, Grassi Jacques, Peoc’h, Katell, Mark W Head, Delisle, Marie Bernadette, Lacroux, C., Lugan Séverine, Schelcher Francois, Delisle Marie Bernadette, Head, Mark W, Ironside James W., Nathalie Streichenberger, Francois Schelcher, Katell Peoch', Perret-Liaudet Armand, Peoch', Katell   +82 more
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Proteomic analysis of the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease [PDF]

, 2007
So far, only the detection of 14-3-3 proteins in cerebrospinal fluid (CSF) has been accepted as diagnostic criterion for Creutzfeldt-Jakob disease (CJD). However, this assay cannot be used for screening because of the high rate of false-positive results,
Steinacker, Petra, Brechlin, Peter, Cepek, Lukas, Klingebiel, Enrico, Bibl, Mirko, Kretzschmar, Hans A., Mollenhauer, Brit, Otto, Markus, Wiltfang, Jens   +8 more
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CSF lactate dehydrogenase activity in patients with Creutzfeldt-Jakob disease exceeds that in other dementias [PDF]

, 2004
The diagnosis of Creutzfeldt- Jakob disease (CJD) is still made by exclusion of other dementias. We now evaluated lactate dehydrogenase (LDH) in the cerebrospinal fluid (CSF) as a possible additional diagnostic tool. CSF LDH levels of patients with CJD (
Cepek, L., Niedmann, P., Poser, S., Otto, M., Kretzschmar, Hans A., Otto, Markus, Poser, Sigrid, Schmidt, H., Schroter, A., Kretzschmar, H. A.   +9 more
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Follow-up investigations of tau protein and S-100B levels in cerebrospinal fluid of patients with Creutzfeldt-Jakob disease [PDF]

, 2005
Background: S-100B and tau protein have a high differential diagnostic potential for the diagnosis of Creutzfeldt-Jakob disease (CJD). So far there has been only limited information available about the dynamics of these parameters in the cerebrospinal ...
Cepek, L., Wiese, Birgitt, Ciesielczyk, Barbara, Cepek, Lukas, Zerr, Inga, Zerr, I., Kretzschmar, Hans A., Poser, Sigrid, Otto, Markus, Steinacker, Petra, Wiltfang, J., Schulz-Schaeffer, Walter J., Wiese, B., Bibl, Mirko, Mollenhauer, Brit, Wiltfang, Jens   +15 more
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Evidence for a pathogenic role of different mutations at codon 188 of PRNP [PDF]

, 2008
Clinical and pathological changes in familial Creutzfeldt-Jakob disease (CJD) cases may be similar or indistinguishable from sporadic CJD. Therefore determination of novel mutations in PRNP remains of major importance.
Eva-Maria Grasbon-Frodl, Hans A Kretzschmar, Roeber, Sigrun, Arzberger, Thomas, Sigrun Roeber, Weber Petra, Krebs, Bjarne, Schröter, Andreas, Petra Weber, Illig, T., Zerr, I., Schröter, A., Arzberger, T., Illig Thomas, Windl, Otto, Bjarne Krebs, Wei Xiang, Hans A. Kretzschmar, Schroeter, Andreas, Kretzschmar, H.A., Inga Zerr, Grasbon-Frodl, Eva-Maria, Weber, Petra, Windl, O., Zerr, Inga, Schröter Andreas, Kretzschmar, Hans A., Xiang, W., Xiang Wei, Illig, Thomas, Zerr Inga, Thomas Illig, Vollmert, C., Grasbon-Frodl Eva-Maria, Kretzschmar Hans A., Caren Vollmert, Weber, P., Krebs Bjarne, Grasbon-Frodl, E.M., Krebs, B., Vollmert Caren, Vollmert, Caren, Roeber Sigrun, Xiang, Wei, Arzberger Thomas, Roeber, S., Windl Otto, Andreas Schröter, Otto Windl, Thomas Arzberger   +49 more
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Creutzfeldt–Jakob disease: A case report and differential diagnoses

Clinical Case Reports, 2022
Although sporadic Creutzfeldt–Jakob disease is a rare neurodegenerative disease and often difficult to diagnose at the earliest onset, meticulous clinical examination, electroencephalography, and neuroimaging findings will help in diagnosis.
Akash Raut, Anjila Thapa, Ashish Shrestha, Kamal Saud, Reema Rajbhandari, Shailendra Katwal   +5 more
doaj   +1 more source