Results 71 to 80 of about 5,104,410 (171)

A Practice Framework for Genetic Testing in Asymptomatic Relatives of Patients With Creutzfeldt‐Jakob Disease: Experience and Insights From Israel

European Journal of Neurology, Volume 33, Issue 6, June 2026.
Presymptomatic genetic testing for genetic Creutzfeldt‐Jakob disease requires careful balancing of autonomy, psychological preparedness, and potential familial implications. Based on multidisciplinary experience in Israel, we propose a structured framework including pre‐test counseling, psychological appraisal, genetic testing, in‐person results ...
Dror Shir, Noa Bregman, Aya Bar David, Talya Nathan, Orly Goldstein, Tanya Gurevich, Avner Thaler, Anat Mirelman, Tamara Shiner, Mali Gana Weisz, Shiri Shkedi‐Rafid, Gael Ganz, Hagit Shani, Elon Pras, Odelia Chorin, Gia Sorkin, Keren Markus‐Bustani, Shlomzion Kahana Merhavi, Mor Shechory‐Stahl, Miri Yanoov Sharav, Michal Dicastro, Dorin Trigubov, Dalit Barel, Esther Kahana, Avi Fellner, Amos Korczyn, Hagit Baris Feldman, Lior Greenbaum, Nurit Omer   +28 more
wiley   +1 more source

A case of probable sporadic Creutzfeldt–Jakob disease

Family Medicine and Community Health, 2017
Creutzfeldt-Jakob Disease (CJD) is a fatal neurodegenerative brain disease. The author describes a case presented to primary care clinic whereby neuropsychiatric symptoms were the patient’s initial presentation which later manifested with declining ...
Norita Hussein
doaj   +1 more source

The Molecular Pathology of Prion Diseases [PDF]

, 2004
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal ...
Vassallo, Neville, Herms, Jochen, Kretzschmar, Hans A.   +2 more
core  

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

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

Sporadic Creutzfeldt-Jakob Disease.

, 2011
The book chapter describes the epidemiology, genetics, clinical features, imaging and laboratory findings, histopathology (including immunohistochemistry and ultrastructure), molecular pathology, subtype classification and differential diagnosis of ...
Head M. W., PARCHI, PIERO, Ironside J. W., Budka H., Gambetti P., Tagliavini F.   +5 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

Sporadic Creutzfeldt–Jakob Disease Appears to Be Sporadic Fatal Insomnia: A Case Report and Review of the Literature

Nature and Science of Sleep, 2021
Qingqing Sun, Pingping Shen, Jiayi Tang, Hongmei Meng, Jiachun Feng, Zan Wang, Li Cui Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, People’s Republic of ChinaCorrespondence: Zan Wang; Li Cui ...
Sun Q, Shen P, Tang J, Meng H, Feng J, Wang Z, Cui L   +6 more
doaj  

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

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