Results 61 to 70 of about 29,749 (223)
Cerebrospinal fluid biomarker supported diagnosis of Creutzfeldt-Jakob disease and rapid dementias: a longitudinal multicentre study over 10 years [PDF]
, 2017 To date, cerebrospinal fluid analysis, particularly protein 14-3-3 testing, presents an important approach in the identification of Creutzfeldt-Jakob disease cases.
Aguzzi, Adriano +20 more
core
Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding [PDF]
, 2010 Transmissible spongiform encephalopathies, or prion diseases, are caused by misfolding and aggregation of the prion protein PrP. These diseases can be hereditary in humans and four of the many disease-associated missense mutants of PrP are in the ...
Daggett, Valerie, van der Kamp, Marc W
core +2 more sources
Ring trial of 2nd generation RT‐QuIC diagnostic tests for sporadic CJD
Annals of Clinical and Translational Neurology, 2020 Objective Real‐time quaking‐induced conversion (RT‐QuIC) assays detect prion‐seeding activity in a variety of human biospecimens, including cerebrospinal fluid and olfactory mucosa swabs.
Christina D. Orrú +21 more
doaj +1 more source
Chemistry – A European Journal, EarlyView.Just as fingerprints reveal identity, spectroscopic fingerprints reveal molecular secrets. In this article, we show parts of the unique fingerprint of the sulfur–aromatic interaction. By measuring how this force alters the electronic structure of gas‐phase peptides, we provide a fundamental understanding of interactions that give insights into the ...
Laura Pille +12 more
wiley +1 more source
Health professions and risk of sporadic Creutzfeldt- Jakob disease, 1965 to 2010 [PDF]
, 2012 In 2009, a pathologist with sporadic Creutzfeldt- Jakob Disease (sCJD) was reported to the Spanish registry. This case prompted a request for information on health-related occupation in sCJD cases from countries participating in the European Creutzfeldt ...
Alcalde-Cabero, E. +22 more
core +2 more sources
Young-onset sporadic Creutzfeldt–Jakob disease with atypical phenotypic features: a case report
Journal of Medical Case Reports, 2019 Background Sporadic Creutzfeldt–Jakob disease, with a mean survival of 6 months, is duly considered among the most fatal neurological disorders. Rapidly progressive dementia with multi-axial involvement of the nervous system is the known presentation ...
Durjoy Lahiri +5 more
doaj +1 more source
Wildlife Letters, EarlyView.We examined changes in perceived risks associated with chronic wasting disease (CWD) and perceived trust in wildlife agencies over time across 10 studies in eight states. Results indicated that perceived risks to both deer and humans declined the longer the disease had been in a state. Results also indicated that agency trust evaluations were positive,
Jerry J. Vaske, Craig A. Miller
wiley +1 more source
Movement Disorders in Prionopathies: A Systematic Review
Tremor and Other Hyperkinetic Movements, 2019 Background: Movement disorders are frequent features of prionopathies. However, their prevalence and onset remain poorly described. Methods: We performed a systematic review of case reports and case series of pathologically- and genetically confirmed ...
Federico Rodriguez-Porcel +11 more
doaj +1 more source
Rapid generation of prion disease models using AAV‐delivered PrP variants in knockout mice
Brain Pathology, EarlyView.We developed a rapid AAV‐based system to generate prion disease models in weeks rather than months. Following systemic AAV9P31 delivery of modified PrP to knockout mice, we achieved brain‐wide expression and successful propagation of both classical (RML) and atypical (GSS‐A117V) prion strains.
Maitena San‐Juan‐Ansoleaga +11 more
wiley +1 more source
Creutzfeldt–Jakob disease: A case report and differential diagnoses
Clinical Case Reports, 2022 Although sporadic Creutzfeldt–Jakob disease is a rare neurodegenerative disease and often difficult to diagnose at the earliest onset, meticulous clinical examination, electroencephalography, and neuroimaging findings will help in diagnosis.
Akash Raut +5 more
doaj +1 more source