Results 71 to 80 of about 6,976 (171)

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

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

Strain Traits of Intracranially Administered L‐Type Bovine Spongiform Encephalopathy Prions Are not Significantly Modified During Intraspecies Transmission in Cynomolgus Monkeys

Microbiology and Immunology, Volume 70, Issue 3, Page 166-174, March 2026.
ABSTRACT Among the three prion strains of bovine spongiform encephalopathy (BSE), classical BSE (C‐BSE) prions are known causative agents of variant Creutzfeldt–Jakob disease. By contrast, human infections with L‐type (L‐) or H‐type (H‐) BSE prions have not been reported.
Ken'ichi Hagiwara, Hiroaki Shibata, Minoru Tobiume, Yuko Sato, Keiko Ohto, Sachi Okabayashi, Nozomi Nakano, Motohiro Horiuchi, Fumiko Ono   +8 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  

Epidemiology of progressive intellectual and neurological deterioration in UK children

Developmental Medicine &Child Neurology, Volume 68, Issue 3, Page 418-428, March 2026.
This study of PIND in UK children was carried out via the British Paediatric Surveillance Unit from 1997 to 2024. It identified six cases of vCJD. 2367 children had other diagnoses explain their deterioration. There were 259 other diseases in the diagnosed group.
Christopher M. Verity, Polly J. Maunder, Anne Marie Winstone, Suvankar Pal   +3 more
wiley   +1 more source

The worst is yet to come: probable sporadic Creutzfeldt–Jakob disease in a well-controlled HIV patient

Prion, 2019
We describe a case of probable sporadic Creutzfeldt–Jakob disease in the setting of well-controlled HIV and discuss whether exist, in fact, HIV-related factors that may predispose to the development of prion disease. To the best of our knowledge, this is
Euripedes Gomes De Carvalho Neto, Matheus Ferreira Gomes, Marina De Oliveira, Maryuris Isabel Niño Guete, Iuri Pereira Santos, Mateus Damiani Monteiro, Fernando Gustavo Stelzer, Fernando Kowacs, Liselotte Menke Barea   +8 more
doaj   +1 more source

Association of genetic predisposition to dyslipidemia and physical activity with incident dementia

Alzheimer's &Dementia, Volume 22, Issue 1, January 2026.
Abstract INTRODUCTION The association between genetic predisposition to dyslipidemia and dementia risk is inconsistent. It remains unknown whether a physically active lifestyle is associated with a lower dementia risk across different levels of this genetic predisposition.
Yumei Liang, Siqi Xie, Dandan Sheng, Lan Ding, Ting Yang, Jianping Jia   +5 more
wiley   +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