Results 51 to 60 of about 16,859 (216)

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
doaj   +1 more source

Prion propagation estimated from brain diffusion MRI is subtype dependent in sporadic Creutzfeldt–Jakob disease

Acta Neuropathologica, 2020
Sporadic Creutzfeldt–Jakob disease (sCJD) is a transmissible brain proteinopathy. Five main clinicopathological subtypes (sCJD-MM(V)1, -MM(V)2C, -MV2K, -VV1, and -VV2) are currently distinguished. Histopathological evidence suggests that the localisation
Riccardo Pascuzzo, N. Oxtoby, A. Young, Janis E. Blevins, G. Castelli, Sara Garbarino, M. Cohen, L. Schonberger, P. Gambetti, B. Appleby, D. Alexander, A. Bizzi   +11 more
semanticscholar   +1 more source

Sporadic Creutzfeldt-Jakob disease in Northern Tasmania

Journal of the Royal College of Physicians of Edinburgh, 2023
Creutzfeldt-Jakob disease is a rare and incurable form of rapidly progressive neurodegenerative disease. The disease is fatal, and most patients die within 1 year of diagnosis. Clinical features include progressive cognitive dysfunction, delusions or hallucinations, cerebellar ataxia, myoclonus, visual disturbances, extrapyramidal signs and eventually
Priyanka Rajalingam, Aaron de Souza, Matthew Lee-Archer, Mahesh Dhakal   +3 more
openaire   +2 more sources

Prions from Sporadic Creutzfeldt-Jakob Disease Patients Propagate as Strain Mixtures

mBio, 2020
sCJD occurrence is currently assumed to result from spontaneous and stochastic formation of a misfolded PrP nucleus in the brains of affected patients. This original nucleus then recruits and converts nascent PrPC into PrPSc, leading to the propagation ...
H. Cassard, Alvina Huor, J. Espinosa, Jean-Yves Douet, S. Lugan, Naima Aron, D. Vilette, M. Delisle, A. Marín-Moreno, P. Péran, V. Béringue, J. Torres, J. Ironside, O. Andréoletti   +13 more
semanticscholar   +1 more source

Movement Disorders in Prionopathies: A Systematic Review

Tremor and Other Hyperkinetic Movements, 2019
Background: Movement disorders are frequent features of prionopathies. However, their prevalence and onset remain poorly described. Methods: We performed a systematic review of case reports and case series of pathologically- and genetically confirmed ...
Federico Rodriguez-Porcel, Vinícius Boaratti Ciarlariello, Alok K. Dwivedi, Lilia Lovera, Gustavo Da Prat, Ricardo Lopez-Castellanos, Ritika Suri, Holly Laub, Ruth H. Walker, Orlando Barsottini, José Luiz Pedroso, Alberto J. Espay   +11 more
doaj   +1 more source

Sporadic Creutzfeldt-Jakob Disease: A Retrospective Analysis of 104 Cases

European Neurology, 2020
Background: Sporadic Creutzfeldt-Jakob disease (sCJD) is an extremely rare fatal and infectious neurodegenerative brain disorder characterized by rapidly progressive dementia, cerebellar ataxia, and visual disturbances.
Chang Qi, Jiatang Zhang, Wei Zhao, X. Xing, Sheng-yuan Yu   +4 more
semanticscholar   +1 more source

Markers of A1 astrocytes stratify to molecular sub-types in sporadic Creutzfeldt–Jakob disease brain

Brain Communications, 2020
Astrocytes are glial cells of the central nervous system that become reactive under conditions of stress. The functional properties of reactive astrocytes depend on their stimulus that induces the upregulation of specific genes. Reactive astrocytes are a
Cathryn L. Ugalde, V. Lewis, C. Stehmann, C. Mclean, V. Lawson, S. Collins, A. Hill   +6 more
semanticscholar   +1 more source

Cerebrospinal fluid real‐time quaking‐induced conversion is a robust and reliable test for sporadic creutzfeldt–jakob disease: An international study

Annals of Neurology, 2016
Real‐time quaking‐induced conversion (RT‐QuIC) has been proposed as a sensitive diagnostic test for sporadic Creutzfeldt–Jakob disease; however, before this assay can be introduced into clinical practice, its reliability and reproducibility need to be ...
L. McGuire, A. Poleggi, I. Poggiolini, Silvia Suardi, Katarina Grznarova, S. Shi, Bart De Vil, S. Sarros, K. Satoh, K. Cheng, M. Cramm, Graham Fairfoul, M. Schmitz, I. Zerr, P. Cras, M. Equestre, F. Tagliavini, Ryuichiro Atarashi, David Knox, S. Collins, S. Haïk, Piero Parchi, M. Pocchiari, A. Green   +23 more
semanticscholar   +1 more source

Enhanced Sensitivity of a Modified Quaking‐Induced Conversion Diagnostic Test for the Broad Detection of Sporadic and Inherited Prion Diseases: A Retrospective Study

Annals of Neurology, EarlyView.
Objective Quaking‐induced conversion (QuIC) tests, which detect prion‐seeding activity in cerebrospinal fluid (CSF), have markedly advanced the antemortem diagnosis of prion diseases such as Creutzfeldt‐Jakob disease (CJD). These tests provide high diagnostic accuracy and enable timely differentiation from other rapidly progressive neurodegenerative ...
Jennifer Myskiw, Rebecca Fox, Dominic M.S. Kielich, Lise Lamoureux, Melanie Leonhardt, Olivia Nykvist, Jessy A. Slota, Kristen Avery, Clark Phillipson, Kathy Frost, Sharon Simon, Brian S. Appleby, Ben A. Bailey‐Elkin, Stephanie A. Booth   +13 more
wiley   +1 more source

Tissue classification for the epidemiological assessment of surgical transmission of sporadic Creutzfeldt-Jakob disease. A proposal on hypothetical risk levels

BMC Public Health, 2005
Background Epidemiological studies on the potential role of surgery in Creutzfeldt-Jakob Disease transmission have disclosed associations with history of specific surgical interventions or reported negative results.
Calero Miguel, Siden Åke, Mølbak Kåre, de Pedro-Cuesta Jesús, Rábano Alberto, Laursen Henning   +5 more
doaj   +1 more source