Results 91 to 100 of about 7,037 (201)

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

Pattern of Cortical Changes in Sporadic Creutzfeldt-Jakob Disease [PDF]

American Journal of Neuroradiology, 2007
High cortical signal intensity on diffusion-weighted (DW) or fluid-attenuated inversion recovery (FLAIR) images is increasingly described in sporadic Creutzfeldt-Jakob disease (sCJD). The aim of this study was to assess the extent and location of high cortical signal intensity, to investigate whether DW or FLAIR is superior in showing changes in ...
Tschampa, Henriette J., Kallenberg, Kai, Kretzschmar, Hans A., Meissner, Bettina, Knauth, Michael, Urbach, Horst, Zerr, I.   +6 more
openaire   +3 more sources

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

A novel form of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: Case report

BMC Neurology, 2010
Background Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder in humans included in the group of Transmissible Spongiform Encephalopathies or prion diseases. The vast majority of sCJD cases are molecularly classified according
Bilbao Miren J, Atarés Begoña, de Pancorbo Marian M, Arteagoitia Jose M, Zarranz Juan J, Garrido Joseba M, Rodríguez-Martínez Ana B, Ferrer Isidro, Juste Ramón A   +8 more
doaj   +1 more source

The presence of valine at residue 129 in human prion protein accelerates amyloid formation

, 2005
The polymorphism at residue 129 of the human PRNP gene modulates disease susceptibility and the clinicopathological phenotypes in human transmissible spongiform encephalopathies.
Tahiri-Alaoui, Abdessamad, Gill, A, Shaw, Michael, Shaw, M, Baskakov, I, Breydo, L, James, W, Tahiri-Alaoui, A, Disterer, Petra, Gill, Andrew, Baskakov, Ilia, Breydo, Leonid, Disterer, P, James, William   +13 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

Application of quantitative DTI metrics in sporadic CJD

NeuroImage: Clinical, 2014
Diffusion Weighted Imaging is extremely important for the diagnosis of probable sporadic Jakob–Creutzfeldt disease, the most common human prion disease.
E. Caverzasi, R.G. Henry, P. Vitali, I.V. Lobach, J. Kornak, S. Bastianello, S.J. DeArmond, B.L. Miller, H.J. Rosen, M.L. Mandelli, M.D. Geschwind   +10 more
doaj   +1 more source

Prion deposition in olfactory biopsy of sporadic Creutzfeldt-Jakob disease.

, 2004
Currently, definite peripheral markers for the in vivo diagnosis of sporadic Creutzfeldt-Jakob disease (CJD) are not available. Here, we report the presence of pathological prion protein in the olfactory mucosa of a case with sporadic Creutzfeldt-Jakob ...
Colucci M., ZANUSSO, Gianluigi, MONACO, Salvatore, Tagliavini F., Cordone M. P., Tabaton M., Giaccone G.   +6 more
core   +1 more source

Epilepsia partialis continua as the presenting manifestation of Creutzfeldt–Jakob disease: A video‐polygraphic clinical vignette

Epileptic Disorders, Volume 28, Issue 3, Page 920-924, June 2026.
Roberta Cutellè, Angelo Pascarella, Ada Santoro, Vittoria Cianci, Anna Mammì, Giovanbattista Gaspare Tripodi, Caterina Prestandrea, Sara Gasparini, Edoardo Ferlazzo   +8 more
wiley   +1 more source

Neuroprotection trial design in progressive supranuclear palsy: challenges and solutions

Alzheimer's &Dementia: Translational Research &Clinical Interventions, Volume 12, Issue 2, April/June 2026.
Abstract INTRODUCTION We review the specific challenges posed to neuroprotective trial design by progressive supranuclear palsy (PSP), a rare disorder difficult to diagnose and to assess quantitatively. METHODS Focusing on reducing the size and cost of trials, we review elements of PSP neuroprotection trial methodology and formulate a new minimum ...
Lawrence I. Golbe, Ronald G. Thomas
wiley   +1 more source