Results 101 to 110 of about 16,859 (216)

Increase of Plasma Biomarkers in Friedreich's Ataxia: Potential Insights into Disease Pathology

Movement Disorders, Volume 40, Issue 9, Page 1863-1873, September 2025.
Abstract Background Therapeutic interventions in Friedreich's ataxia (FRDA) are progressing into clinical trials, and the need for robust and easily accessible biomarkers has arisen. Objective This study aimed to consolidate preliminary findings of changes in the levels of neurofilament light (NfL), glial fibrillary acidic protein (GFAP), Tau, and ...
Christian Rummey, Gilbert Thomas‐Black, Hector Garcia‐Moreno, David R. Lynch, Rosella Abeti, Huseyin Arisoy, Amanda Heslegrave, Henrik Zetterberg, Paola Giunti, the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS), Jörg Bernhard Schulz, Kathrin Reetz, Claire Didszun, Thomas Klockgether, Ilaria Giordano, Massimo Pandolfo, Chantal Depondt, Myriam Rai, Sylvia Boesch, Wolfgang Nachbauer, Andreas Eigentler, Elisabetta Indelicato, Paola Giunti, Michael Parkinson, Katarina Manso, Gilbert Thomas‐Black, Hector Garcia‐Moreno, Nita Solanky, Rosella Abeti, James Polke, Robin Labrum, Francisco Rodríguez de Rivera Garrido, Javier Mascias, Sara Sánchez Velasco, Sergio Secades García, Caterina Mariotti, Lorenzo Nanetti, Anna Castaldo, Alessia Mongelli, Mario Fichera, Thomas Klopstock, Ivan Karin, Claudia Stendel, Florentine Radelfahr, Alexandra Durr, Perrine Charles, Claire Ewenczyk, Jennifer Just, Georgios Koutsis, Richard Walsh, Enrico Bertini   +50 more
wiley   +1 more source

The new variant of the Creutzfeldt-Jakob disease accounts for no relative increase of the Creutzfeldt-Jakob disease mortality rate in the United Kingdom; this fits ill with the new variant being the consequence of consumption of food infected with the agent of Bovine Spongiform Encephalopathy

BMC Public Health, 2003
Background A new variant of Creutzfeldt-Jakob disease was described in the United Kingdom. It is often claimed that it is caused by consumption of food infected with the agent of bovine spongiform encephalopathy.
Hénaut Alain, Laprevotte Ivan
doaj   +1 more source

MRI lesion profiles in sporadic Creutzfeldt–Jakob disease

Neurology, 2009
With respect to sporadic Creutzfeldt-Jakob disease (sCJD), six molecular subtypes (MM1, MM2, MV1, MV2, VV1, and VV2) have been described, which vary with respect to age at disease onset, disease duration, early symptoms, and neuropathology. MRI signal alterations were reported to correlate with distinct Creutzfeldt-Jakob disease (CJD) subtypes.
Meissner, B., Kallenberg, K., Sanchez-Juan, P., Collie, D., Summers, D. M., Almonti, S., Collins, S. J., Smith, P., Cras, P., Jansen, G. H., Brandel, J. P., Coulthart, M. B., Roberts, H., van Everbroeck, B., Galanaud, Damien, Mellina, V., Will, R. G., Zerr, I.   +17 more
openaire   +5 more sources

Pathological spectrum of sporadic Creutzfeldt–Jakob disease

Pathology
Human prion diseases are a rare group of transmissible neurodegenerative conditions which are classified according to their aetiology as sporadic, genetic or acquired forms. Creutzfeldt-Jakob disease (CJD) is the most common form of human prion disease, with the sporadic form accounting for ∼85% of all reported cases.
Diane L, Ritchie, Colin, Smith
openaire   +2 more sources

Prion-related peripheral neuropathy in sporadic Creutzfeldt-Jakob disease

Journal of Neurology Neurosurgery & Psychiatry, 2018
Objective To assess whether the involvement of the peripheral nervous system (PNS) belongs to the phenotypic spectrum of sporadic Creutzfeldt-Jakob disease (sCJD). Methods We examined medical records of 117 sCJDVV2 (ataxic type), 65 sCJDMV2K (kuru-plaque
Simone Baiardi, V. Redaelli, P. Ripellino, Marcello Rossi, A. Franceschini, M. Moggio, P. Sola, A. Ladogana, P. Fociani, A. Magherini, S. Capellari, A. Giese, B. Caughey, P. Caroppo, Piero Parchi   +14 more
semanticscholar   +1 more source

Prion disease diagnosis using subject-specific imaging biomarkers within a multi-kernel Gaussian process

NeuroImage: Clinical, 2019
Prion diseases are a group of rare neurodegenerative conditions characterised by a high rate of progression and highly heterogeneous phenotypes. Whilst the most common form of prion disease occurs sporadically (sporadic Creutzfeldt–Jakob disease, sCJD ...
Liane S. Canas, Carole H. Sudre, Enrico De Vita, Akin Nihat, Tze How Mok, Catherine F. Slattery, Ross W. Paterson, Alexander J.M. Foulkes, Harpreet Hyare, M. Jorge Cardoso, John Thornton, Jonathan M. Schott, Frederik Barkhof, John Collinge, Sébastien Ourselin, Simon Mead, Marc Modat   +16 more
doaj   +1 more source

Sporadic Creutzfeldt-Jakob disease

Neurology, 2004
To assess if clinical features, prion protein codon 129, and molecular subtype correlate with MRI basal ganglia hyperintensity in sporadic Creutzfeldt-Jakob disease (CJD).The authors studied 219 patients including 153 confirmed CJD cases for their neurologic symptoms and MRI findings.
Meissner, Bettina, Kohler, K., Kortner, K., Bartl, Mario, Jastrow, U., Mollenhauer, Brit, Schroter, A., Finkenstaedt, M., Windl, Otto, Poser, Sigrid, Kretzschmar, Hans A., Zerr, I.   +11 more
openaire   +3 more sources

Cerebrospinal Fluid Markers in Sporadic Creutzfeldt-Jakob Disease

International Journal of Molecular Sciences, 2011
Sporadic Creutzfeldt-Jakob disease (sCJD) is the commonest form of human prion diseases, accounting for about 85% of all cases. Current criteria for intra vitam diagnosis include a distinct phenotype, periodic sharp and slow-wave complexes at electroencephalography (EEG), and a positive 14-3-3-protein assay in the cerebrospinal fluid (CSF).
ZANUSSO, Gianluigi, FIORINI, Michele, FERRARI, Sergio, GAJOFATTO, Alberto, Cagnin A., Galassi A., Richelli S., MONACO, Salvatore   +7 more
openaire   +3 more sources

Detection of Creutzfeldt-Jakob disease prions in skin: implications for healthcare

Genome Medicine, 2018
Editorial summary Evidence has recently been reported of prion seeding activity in skin tissue from patients with sporadic Creutzfeldt-Jakob disease (sCJD). This is relevant information for infection control measures during surgery.
Akin Nihat, Simon Mead
doaj   +1 more source

Creutzfeldt–Jakob disease‐like imaging in valosin‐containing protein D395G mutation

Alzheimer's &Dementia, Volume 21, Issue 10, October 2025.
Ryota Kobayashi, Masafumi Kanoto, Shinobu Kawakatsu, Akihito Suzuki   +3 more
wiley   +1 more source