Results 61 to 70 of about 11,518 (218)

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, Volume 99, Issue 5, Page 1303-1314, May 2026.
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

Probable sporadic Creutzfeldt–Jakob disease mimicking focal epilepsy

Epilepsy and Behavior Case Reports, 2019
Creutzfeldt–Jakob disease (CJD) presents with seizures as an early symptom in only approximately 3% of cases. These seizures often present as nonconvulsive status epilepticus (NCSE) or epilepsia partialis continua (EPC).
Pei-Shan Hsiao, Yuan-Ming Lee, Fu-Sin Chu, Chao-Lin Lee, Fang-Chun Liu, Ping-Huang Tsai   +5 more
doaj   +1 more source

Distinct origins of dura mater graft-associated Creutzfeldt-Jakob disease: past and future problems [PDF]

, 2014
Dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) can be divided into two subgroups that exhibit distinct clinical and neuropathological features, with the majority represented by a non-plaque-type of dCJD (np-dCJD) and the minority by a ...
Atsushi Kobayashi, Shirou Mohri, Tetsuyuki Kitamoto, Yuichi Matsuura   +3 more
core   +1 more source

Evidence for different seeding activities of misfolded tau in classical and rapidly progressive Alzheimer's disease

Brain Pathology, Volume 36, Issue 3, May 2026.
The study analyzed the seeding activity of misfolded tau protein in Alzheimer's disease and rapidly progressive Alzheimer's disease patients and provides evidence for the existence of different tau assemblies supported by differences in cellular toxicity and morphology of thioflavin T‐positive real‐time quaking‐induced conversion products.
Matthias Schmitz, Katrin Thüne, Anna‐Lisa Fischer, Peter Hermann, Maria Cramm, Leonie Sheila Kadel, Wiebke Möbius, Jakob Matschke, Tayyaba Saleem, Neelam Younas, Saima Zafar, Hermann Altmeppen, Markus Glatzel, Inga Zerr   +13 more
wiley   +1 more source

ENFERMEDAD DE JAKOB-CREUTZFELDT. A PROPÓSITO DE UN CASO

Revistas Argentina de Medicina, 2017
La enfermedad de Jakob-Creutzfeuldt esporádica es la más común de las patologías priónicas humanas. Se presenta el caso de una paciente de 59 años de edad internada por trastornos conductuales cognitivos, acompañados de mioclonías y mutismo acinético.
Lucía González, José Fernández, Vanesa Aguilar, Silvana Tobares, Melisa Contreras, Livio Lanciani, Jimena Boccardo, Juan Carlos Giugni   +7 more
doaj   +2 more sources

Imaging and CSF analyses effectively distinguish CJD from its mimics [PDF]

, 2017
OBJECTIVE: To review clinical and investigation findings in patients referred to a specialist prion clinic who were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to have an alternative final diagnosis. METHODS: Review the
Collinge, J, Green, A, Hyare, H, Mead, S, Rudge, P   +4 more
core   +1 more source

Variably Protease‐Sensitive Prionopathy: Two New Cases With Motor Neuron‐Dementia Syndrome

Annals of Clinical and Translational Neurology, Volume 13, Issue 4, Page 827-833, April 2026.
ABSTRACT We describe two patients with variably protease‐sensitive prionopathy (VPSPr) who developed progressive upper motor neuron symptoms, insomnia, behavioral and cognitive decline, compatible with primary lateral sclerosis associated with frontotemporal dementia (FTD).
María Elena Erro, María Victoria Zelaya, Hasier Eraña, Javier Sánchez Ruiz de Gordoa, Fermín García‐Amigot, Anika Simonovska‐Serra, María Cristina Caballero, Isidre Ferrer, Ellen Gelpi, Ivonne Jericó, Joaquín Castilla   +10 more
wiley   +1 more source

Distribution of microRNA profiles in pre-clinical and clinical forms of murine and human prion disease

Communications Biology, 2021
Cheng et al. present differentially expressed extracellular vesicle miRNAs in human clinical samples from patients who suffer from sporadic Creutzfeldt-Jakob disease.
Lesley Cheng, Camelia Quek, Xia Li, Shayne A. Bellingham, Laura J. Ellett, Mitch Shambrook, Saima Zafar, Inga Zerr, Victoria A. Lawson, Andrew F. Hill   +9 more
doaj   +1 more source

Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years [PDF]

, 2015
Patients with iatrogenic Creutzfeldt-Jakob disease due to administration of cadaver-sourced growth hormone during childhood are still being seen in the UK 30 years after cessation of this treatment.
Adlard, P, Bjurstrom, N, Brandner, S, Caine, D, Collinge, J, Druyeh, R, Hummerich, H, Hyare, H, Jaunmuktane, Z, Lowe, J, Mead, S, Norsworthy, P, Rudge, P, Wadsworth, JD   +13 more
core   +1 more source

A novel phenotype of sporadic Creutzfeldt–Jakob disease [PDF]

BMJ Case Reports, 2007
An atypical case of sporadic Creutzfeldt–Jakob disease (CJD) is described in a 78-year-old woman homozygous for methionine at codon 129 of the prion protein (PrP) gene. The neuropathological signature was the presence of PrP immunoreactive plaque-like deposits in the cerebral cortex, striatum and thalamus.
G, Giaccone, G, Di Fede, M, Mangieri, L, Limido, R, Capobianco, S, Suardi, M, Grisoli, S, Binelli, P, Fociani, O, Bugiani, F, Tagliavini   +10 more
openaire   +4 more sources