Results 91 to 100 of about 11,518 (218)

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

Sporadic Creutzfeldt-Jakob disease. Case report

São Paulo Medical Journal, 2021
Context: Creutzfeldt-Jakob disease (CJD) is a rare disease that belongs to the category of transmissible spongiform encephalopathies. The condition is invariably fatal and progresses with severe dementia with psychiatric signs and, with cortical, subcortical and cerebellar signs.
Joseph Bruno Bidin Brooks, Fábio César Prosdócimi, Fernanda Stoffel Covolan, Iane Rocha Holanda, Amanda Medeiros de Lucena, Eduarda Mendes Prado Macedo, Bianca Angerami de Souza Albero, Lucas Veiga Guimaraes   +7 more
openaire   +1 more source

Peripheral Signatures of Multidimensional Pathology in Symptomatic and Asymptomatic Creutzfeldt–Jakob Disease

CNS Neuroscience &Therapeutics, Volume 32, Issue 1, January 2026.
Plasma biomarkers in CJD reveal multisystem involvement, with neuronal injury markers showing strong discriminative performance and vascular proteins indicating blood–brain barrier dysfunction. In asymptomatic PRNP mutation carriers, biomarker changes are minimal and emerge mainly near disease onset ABSTRACT Background Plasma markers of neuronal injury
Zhong‐Yun Chen, Min Chu, Yi‐Hao Wang, Rui Liu, Jing Zhang, Ai‐ling Yue, Hua Lu, Qian‐qian He, Jia‐hui Hou, Yu‐fei Chen, Hong Ye, Li‐Yong Wu   +11 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

Does Improvement in Case Ascertainment Explain the Increase in Sporadic Creutzfeldt-Jakob Disease Since 1970 in the United Kingdom? [PDF]

, 2017
The aim of this study was to quantify the improvements in case ascertainment which are considered to explain the rise in the incidence of sporadic Creutzfeldt-Jakob disease.
Cohen, Carine H.
core  

Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA [PDF]

, 2012
The current classification of human sporadic prion diseases recognizes six major phenotypic subtypes with distinctive clinicopathological features, which largely correlate at the molecular level with the genotype at the polymorphic codon 129 (methionine,
Cohen, Mark L, de Boni, Laura, Ferrer, Isidro, Gambetti, Pierluigi, Gelpi, Ellen, Giaccone, Giorgio, Giese, Armin, Hauw, Jean-Jacques, Höftberger, Romana, Ironside, James W, Jansen, Casper, Kovacs, Gabor G, Kretzschmar, Hans A, Parchi, Piero, Rozemuller, Annemieke, Saverioni, Daniela, Seilhean, Danielle, Tagliavini, Fabrizio   +17 more
core   +1 more source

Incidence of Creutzfeldt‐Jakob Disease in a Tertiary Care Referral Center in Mexico City

Alzheimer's &Dementia, Volume 21, Issue S6, December 2025.
Abstract Background Creutzfeldt‐Jakob disease (CJD) is a progressive, irreversible, and fatal disease associated with the misfolding of a protein in the central nervous system. Several surveillance programs emerged worldwide after the epidemic of the early 2000s. In Mexico, information about its epidemiology is scarce.
Ilse Murrieta Hernandez, Enrique Isaac Tetlalmatzi Azuara, Hector Tellez Lucero, Juan Carlos Lopez Hernandez, Enrique Piña Rosales, Brenda Dzul Garcia, Raul Medina Rioja   +6 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  

MRI and clinical syndrome in dura materrelated Creutzfeldt-Jakob disease [PDF]

, 2018
Objective : Iatrogenic Creutzfeldt-Jakob disease (iCJD) is mainly associated with dura mater (DM) grafts and administration of human growth hormones (hGH). Data on disease course in DM-CJD are limited.
Barsic, B., Eigenbrod, S., Gelpi, E., Heinemann, U., Kallenberg, K., Knauth, M., Krasnianski, A., Kretzschmar, H., Meissner, B., Ramljak, S., Sanchez-Juan, P., Schulz-Schaeffer, W., Zerr, I.   +12 more
core  

Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance [PDF]

, 2013
BACKGROUND: There is increasing epidemiological evidence of etiological links between general surgery and sporadic Creutzfeldt-Jakob disease (sCJD) with long incubation periods.
Ake Siden, Alberto Rábano, Henning Laursen, Ignacio Mahillo-Fernandez, Javier Almazán, Jesus de Pedro-Cuesta, Kåre Mølbak, Mabel Cruz, Miguel Calero, null null   +9 more
core   +3 more sources