Results 61 to 70 of about 26,898 (212)

The Phenotypic Spectrum of Sporadic Creutzfeldt‐Jakob Disease Cortical Subtype

Annals of Neurology, EarlyView.
Objective The objective of this study was to characterize the phenotypic spectrum of the rare sporadic Creutzfeldt‐Jakob disease cortical subtype (sCJDMM/MV2C) in a large multicentric autopsy cohort. Methods We evaluated clinical histories, biofluid markers, brain diffusion‐weighted (DW)‐magnetic resonance imaging (MRI), and electroencephalogram (EEG ...
Simone Baiardi, Claudia Marina Vargiu, Brian S. Appleby, Marcelo Barria, Giuseppe Mario Bentivenga, Ignazio Calì, Benedetta Carlà, Mark Cohen, Armin Giese, Jochen Herms, Aino‐Minerva Kortelainen, Anna Ladogana, Angela Mammana, Diane Ritchie, Otto Windl, Sabina Capellari, Piero Parchi   +16 more
wiley   +1 more source

MicroRNAs in Prion Diseases—From Molecular Mechanisms to Insights in Translational Medicine

Cells, 2021
MicroRNAs (miRNAs) are small non-coding RNA molecules able to post-transcriptionally regulate gene expression via base-pairing with partially complementary sequences of target transcripts.
Danyel Fernandes Contiliani, Yasmin de Araújo Ribeiro, Vitor Nolasco de Moraes, Tiago Campos Pereira   +3 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, EarlyView.
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

Infection Risk From Humans and Animals in the Anatomy Laboratory: A Scoping Review

Clinical Anatomy, EarlyView.
ABSTRACT Whole‐body dissection is a cornerstone of anatomy education. During and following the COVID‐19 pandemic, exposure to infectious agents and other risks of dissection were highlighted. To identify potential risks, one must have the data outlining these risks in specific situations.
Margaret A. McNulty, Elizabeth R. Agosto
wiley   +1 more source

Molecular dynamics as an approach to study prion protein misfolding and the effect of pathogenic mutations [PDF]

, 2011
Computer simulation of protein dynamics offers unique high-resolution information that complements experiment. Using experimentally derived structures of the natively folded prion protein (PrP), physically realistic dynamics and conformational changes ...
Daggett, Valerie, van der Kamp, Marc W
core   +2 more sources

Implant Therapy in Patients With Neurodegenerative Diseases—A Scoping Review

Clinical Oral Implants Research, EarlyView.
ABSTRACT Objectives The increasing longevity of populations has resulted in a growing number of older adults requiring prosthodontic care, including those with neurodegenerative diseases (NDs). Neurodegenerative diseases pose significant challenges to prosthodontic care, and it remains unclear whether implant therapy in this population achieves ...
Lysandre David, Sabrina Maniewicz, Najla Chebib, Gabriel Gold, Murali Srinivasan, Frauke Müller   +5 more
wiley   +1 more source

Human stem cell-derived astrocytes replicate human prions in a PRNP genotype-dependent manner. [PDF]

, 2017
Prions are infectious agents that cause neurodegenerative diseases such as Creutzfeldt-Jakob disease (CJD). The absence of a human cell culture model that replicates human prions has hampered prion disease research for decades.
Alibhai, James, Chandran, Siddharthan, Head, Mark W, Ironside, James W, Krejciova, Zuzana, Krencik, Robert, Manson, Jean, Rzechorzek, Nina M, Ullian, Erik M, Zhao, Chen   +9 more
core   +1 more source

Epidemiology of progressive intellectual and neurological deterioration in UK children

Developmental Medicine &Child Neurology, EarlyView.
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

Defining the Prion Type of Fatal Familial Insomnia

Pathogens, 2021
Fatal familial insomnia (FFI) belongs to the genetic human transmissible spongiform encephalopathies (TSE), such as genetic Creutzfeldt-Jakob disease (CJD) or Gerstmann-Straeussler-Scheinker syndrome (GSS).
Wiebke Jürgens-Wemheuer, Arne Wrede, Walter Schulz-Schaeffer   +2 more
doaj   +1 more source

Neuroimagen estructural y funcional en las enfermedades priónicas humanas [PDF]

, 2011
INTRODUCTION: Prion diseases are neurodegenerative disorders resulting from the accumulation of a misfolded isoform of the cellular prion protein (PrPc). They can occur as acquired, sporadic or hereditary forms. Although prion diseases show a wide range
Arbizu, J. (Javier), Carmona-Abellan, M. (M.), Dominguez-Prado, I. (Inés), Luquin, M.R. (María Rosario), Ortega-Cubero, S. (Sara), Pagola, I. (I.), Riverol, M. (Mario)   +6 more
core