Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease [PDF]
Emerging Infectious Diseases, 2014 We report the presence of infectivity in erythrocytes, leukocytes, and plasma of 1 person with variant Creutzfeldt-Jakob disease and in the plasma of 2 in 4 persons whose tests were positive for sporadic Creutzfeldt-Jakob disease.
Jean Yves Douet +11 more
doaj +2 more sources
Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease [PDF]
, 2021 Sporadic Creutzfeldt-Jakob disease is a fatal neurodegenerative disease caused by misfolded prion proteins (PrP^{Sc}). Effective therapeutics are currently not available and accurate diagnosis can be challenging.
Kovacs, GG +18 more
core +5 more sources
Diagnostic approach in a patient with Creutzfeldt-Jakob disease [PDF]
Dementia & Neuropsychologia, 2022 Prion diseases are an important cause of rapidly progressive dementias. Among them, the most common is sporadic Creutzfeldt-Jakob disease (CJD). It is a rare and incurable disease, with rapid progression to death.
José Wagner Leonel Tavares-Júnior +9 more
doaj +1 more source
Characterization of Prion Disease Associated with a Two-Octapeptide Repeat Insertion
Viruses, 2021 Genetic prion disease accounts for 10–15% of prion disease. While insertion of four or more octapeptide repeats are clearly pathogenic, smaller repeat insertions have an unclear pathogenicity.
Nicholas Brennecke +13 more
doaj +1 more source
The human spongiform encephalopathies [PDF]
Romanian Journal of Neurology, 2018 The human spongiform encephalopathies are a group of heterogenous, usually fatal diseases, characterized by a unique pathogenetic mechanism and distinct clinical presentation. They are classified into sporadic, familial and acquired forms.
Mavroudis Ioannis +2 more
doaj +1 more source
Sporadic Creutzfeldt-Jakob disease with cerebellar ataxia at onset in the UK [PDF]
, 2006 To determine the frequency, in the UK, of sporadic Creutzfeldt-Jakob Disease (sCJD) with a cerebellar ataxic onset, and to describe the clinical features of the ...
Will, R G +4 more
core +1 more source
Validation of the 2017 International Creutzfeldt-Jakob Disease Surveillance Network diagnostic criteria for sporadic Creutzfeldt-Jakob disease [PDF]
, 2023 Sporadic Creutzfeldt-Jakob disease (sCJD) is the commonest human prion disease. sCJD is rapidly-progressive, universally fatal and transmissible.
Watson, Neil Robert John
core +1 more source
Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification [PDF]
, 2009 Six subtypes of sporadic Creutzfeldt-Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrPSc, and the methionine (M)/valine (V) polymorphic codon 129 of the prion ...
Maurizio Pocchiari +51 more
core +1 more source
Annals of Clinical and Translational Neurology, 2023 Beta‐synuclein is a promising cerebrospinal fluid and blood biomarker of synaptic damage. Here we analysed its accuracy in the discrimination between sporadic Creutzfeldt–Jakob disease (n = 150) and non‐prion rapidly progressive dementias (n = 106).
Samir Abu‐Rumeileh +11 more
doaj +1 more source
Efficient transmission and characterization of creutzfeldt-jakob disease strains in bank voles. [PDF]
, 2006 Transmission of prions between species is limited by the "species barrier," which hampers a full characterization of human prion strains in the mouse model.
Bari Michele A. Di +44 more
core +1 more source