|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
The existence of affected subjects with both abnormal prion protein (PrP<sup>Sc</sup>) types has been reported, and their clinical features were somewhat similar to the dominant PrP<sup>Sc</sup> type but varied in sporadic Creutzfeldt-Jakob disease (sCJD).
|
31675505 |
2020 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Compared to ND, t-PrP concentrations were significantly decreased in sCJD, iCJD and in genetic prion diseases associated with the three most common mutations E200K, V210I (associated with genetic CJD) and D178N-129M (associated with fatal familial insomnia).
|
30062673 |
2019 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
This information may be used with clinical measures to inform the progression of sCJD and patterns of prion protein spread throughout the brain.
|
30735286 |
2019 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
The diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) can only be confirmed by abnormal protease-resistant prion protein accumulation in post-mortem brain tissue.
|
31306078 |
2019 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
The clinical presentation in Chinese patients with sporadic Creutzfeldt-Jakob disease (sCJD) may be unique due to the big difference in the codon 129 polymorphism of the prion protein gene (PRNP).
|
31651418 |
2019 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Sporadic Creutzfeldt-Jakob Disease (CJD) is the most common human prion disease, accounting for approximately 85-90% of cases, whereas autosomal dominant genetic forms, due to mutations in the prion protein gene (PRNP), account for 10-15% of cases.
|
30606247 |
2019 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
The transmissible agent linked to sCJD is composed of the misfolded form of the host-encoded prion protein.
|
31206560 |
2019 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
This case highlights the clinical heterogeneity of sCJD presentation and the important inclusion of CJD in the differential diagnosis of atypical presentations of neurodegenerative disease.
|
30850568 |
2019 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
The cerebellar lesions of bovine spongiform encephalopathy (BSE)-infected guinea pigs were characterized as severe atrophy of the cerebellar cortex associated with the loss of granule cells, decrease in the width of the molecular layer, and intense protease-resistant prion protein (PrP<sup>Sc</sup> ) accumulations that are similar to cerebellar lesions in kuru and the VV2 type of sporadic Creutzfeldt-Jakob disease.
|
31797465 |
2019 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Herein, we detected CCD in five patients with sCJD and four patients with genetic CJD through SPECT.
|
30308445 |
2018 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
We report the synthesis of new human prion from the recombinant human prion protein expressed in bacteria in reaction seeded with sCJD MM1 prions and cofactor, ganglioside GM1.
|
29867164 |
2018 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
We studied the amount/severity and distribution of activated microglia, protease-resistant prion protein (PrP<sup>Sc</sup> ) spongiform change, and astrogliosis in eight regions of 57 brains, representative of the entire spectrum of sCJD subtypes.
|
29345730 |
2018 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
Proteinase K-resistant prion protein (PrP<sup>Res</sup> ) nuclear and perinuclear immunoreactivity in oligodendrocytes of the frontal cortex is found in one case of otherwise typical sporadic Creutzfeldt-Jakob disease (sCJD) type VV2a.
|
30123962 |
2018 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
PMCA shows great potential for the diagnosis of variant CJD (vCJD) and has the ability to distinguish vCJD from sCJD, which may become increasingly important with emergence of a patient with neuropathologically confirmed vCJD associated with PRNP codon129MV, which indicates that a new wave of vCJD cases is likely and that these may be difficult to distinguish from sCJD.
|
29887145 |
2018 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
We immunostained the frontal cortex, basal ganglia and cerebellum in 16 patients with rpAD and sCJD using antibodies against markers of microglia and recruited monocytes (ionized calcium-binding adaptor molecule 1, human leukocyte antigen DPQR, Cluster of Differentiation 68), an antibody unique to brain-resident microglia (transmembrane protein 119 (TMEM119)), in addition to antibodies against a marker of astrocytes (glial fibrillary acidic protein), amyloid-β (Aβ) and pathological prion protein. rpAD patients showed a distinct microglial phenotype with a high abundance of TMEM119-positive microglia in all investigated regions.
|
30318820 |
2018 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
In the framework of a prospective epidemiologic study, all neuropathologically confirmed cases with sCJD who received CSF RT-QuIC analysis during diagnostic workup (n = 65) and a control group of individuals without CJD (n = 118) were selected to investigate the accuracy of an amended diagnostic protocol.
|
29934424 |
2018 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
Diagnosis of genetic, variant, and iatrogenic CJD was ruled out based on the mother's clinical history, genetic tests, and biochemical investigations, all of which supported the diagnosis of sCJD.
|
29889261 |
2018 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Sporadic Creutzfeldt-Jakob disease (CJD), the most common human prion disease, is generally regarded as a spontaneous neurodegenerative illness, arising either from a spontaneous PRNP somatic mutation or a stochastic PrP structural change.
|
29887134 |
2018 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
In comparison to sporadic Creutzfeldt-Jakob disease (sCJD) with MM1-type and MM2- cortical (MM2C)-type, genetic CJD with a prion protein gene V180I mutation (V180I gCJD) is clinically characterized by onset at an older age, slower progress, and the absence of visual disturbances or cerebellar symptoms.
|
29264994 |
2018 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Sporadic Creutzfeldt-Jakob disease (sCJD), the most common human prion disease, is transmissible through iatrogenic routes due to abundant infectious prions [misfolded forms of the prion protein (PrP<sup>Sc</sup>)] in the central nervous system (CNS).
|
29167394 |
2017 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
Amyloid- and FDG-PET in sporadic Creutzfeldt-Jakob disease: Correlation with pathological prion protein in neuropathology.
|
28509609 |
2017 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
Sporadic Creutzfeldt-Jakob disease (sCJD) is the most prevalent form of human prion disease and it is characterized by the presence of neuronal loss, spongiform degeneration, chronic inflammation and the accumulation of misfolded and pathogenic prion protein (PrP<sup>Sc</sup>).
|
28449707 |
2017 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
The polymorphism at codon 129 of the prion protein gene (PRNP) and the PrPSc types 1 and 2 belong to a molecular classification of sporadic Creutzfeldt-Jakob disease (sCJD) that correlates well with the clinical and neuropathological phenotype of sCJD.
|
28598840 |
2017 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
The majority of cases of human prion diseases occur worldwide in the form of sporadic Creutzfeldt-Jakob disease and a minority of around 10-15% are associated with mutations of the prion protein gene, termed PRNP, in the forms of genetic Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia.
|
28987186 |
2017 |
|
Creutzfeldt-Jakob Disease, Sporadic
|
0.400 |
Biomarker
|
disease |
BEFREE |
A causal link between the treatment with plasma products and the development of sCJD has not been established, and the occurrence of these cases may simply reflect a chance event in the context of systematic surveillance for CJD in large populations.
|
28516863 |
2017 |