Results 71 to 80 of about 16,859 (216)

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

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

Structural signature of sporadic Creutzfeldt–Jakob disease

European Journal of Neurology, 2019
Background and purposeSporadic Creutzfeldt–Jakob disease (sCJD) is a rapidly progressive neurodegenerative disease caused by an abnormal isoform of the human prion protein. Structural magnetic resonance imaging in patients with pathologically confirmed sCJD was compared with cognitively normal individuals to identify a cortical thickness signature of ...
J. Navid, G. S. Day, J. Strain, R. J. Perrin, R. C. Bucelli, A. Dincer, J. K. Wisch, D. Soleimani‐Meigooni, J. C. Morris, T. L. S. Benzinger, B. M. Ances   +10 more
openaire   +6 more sources

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

Premortem diagnosis of pathologically confirmed sporadic Creutzfeldt‐Jakob disease

Clinical Case Reports, 2021
Sporadic Creutzfeldt‐Jakob disease should be considered in any case of rapid neuropsychiatric decline. While neuropathological examination of a brain biopsy specimen remains the only definitive diagnostic method and real‐time quaking‐induced conversion ...
Elina T Ziukelis, Vasu Keshav Sharma, James J Gome   +2 more
doaj   +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

Human transmissible spongiform encephalopathies in eleven countries: diagnostic pattern across time, 1993–2002

BMC Public Health, 2006
Background The objective of this study was to describe the diagnostic panorama of human transmissible spongiform encephalopathies across 11 countries. Methods From data collected for surveillance purposes, we describe annual proportions of deaths due to ...
Jansen Gerard H, Lewis Victoria, Collins Steven, Sanchez-Juan Pascual, Van Duijn Cornelia, Alpérovitch Annick, Delasnerie-Lauprêtre Nicole, Brandel Jean-Philippe, Kretszchmar Hans A, Zerr Inga, Pocchiari Maurizio, Puopolo Maria, Mellina Vittorio, Stoeck Katharina, Almazán Javier, Glatzel Markus, de Pedro-Cuesta Jesús, Coulthart Michael B, Gelpi Ellen, Budka Herbert, Mitrova Eva   +20 more
doaj   +1 more source

Anti-N-methyl-D-Aspartate Receptor Encephalitis Mimicking Sporadic Creutzfeldt–Jakob Disease

Frontiers in Neurology, 2020
Objectives: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and sporadic Creutzfeldt–Jakob disease (sCJD) share similar clinical features. Here, we present two unusual cases of anti-NMDAR encephalitis who were misdiagnosed as sCJD at first ...
Jiao Liu, Liyan Chen, Jing Yang, Lan Wang, Huifang Shang, Xueping Chen   +5 more
doaj   +1 more source

Is sporadic Creutzfeldt‐Jakob disease transfusion‐transmissible? [PDF]

Transfusion, 2020
See article on page 694–697, in this ...
openaire   +2 more sources

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