Interlaboratory validation of cerebrospinal fluid α‐synuclein quantification in the diagnosis of sporadic Creutzfeldt‐Jakob disease [PDF]
Alzheimer’s & Dementia: Diagnosis, Assessment & Disease Monitoring, 2018 Introduction Cerebrospinal fluid α‐synuclein level is increased in sporadic Creutzfeldt‐Jakob disease cases. However, the clinical value of this biomarker remains to be established.
Niels Kruse +12 more
doaj +2 more sources
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
Probable sporadic Creutzfeldt–Jakob disease mimicking focal epilepsy
Epilepsy and Behavior Case Reports, 2019 Creutzfeldt–Jakob disease (CJD) presents with seizures as an early symptom in only approximately 3% of cases. These seizures often present as nonconvulsive status epilepticus (NCSE) or epilepsia partialis continua (EPC).
Pei-Shan Hsiao +5 more
doaj +2 more sources
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 +2 more
doaj +2 more sources
Understanding the Phenotypic Heterogeneity Within the Sporadic Creutzfeldt-Jakob Disease MV1 Subtype. [PDF]
Neuropathol Appl NeurobiolWe present the clinical, pathological and prion biophysical features of three atypical cases of the MV1 subtype of sporadic Creutzfeldt–Jakob disease (sCJD). We propose that these heterozygous cases exist on a spectrum ranging from MM1‐like to VV1‐like phenotypes and recommend that subtyping be performed at pH 6.9 to avoid missing atypical or mixed ...
Nemani SK +6 more
europepmc +2 more sources
Sporadic Creutzfeldt-Jakob disease: Clinical, pathological and molecular study [PDF]
Revista Ciencias de la Salud, 2008 phalopathiesare neurodegenerative diseasescaused by abnormal accumulation of pathogenicisoform the prion protein, which induces theformation of conglomerates protein resistantto degradation.
Victoria Eugenia Villegas +2 more
doaj +1 more source
The Phenotypic Spectrum of Sporadic Creutzfeldt-Jakob Disease Cortical Subtype. [PDF]
Ann NeurolObjective 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 ...
Baiardi S +16 more
europepmc +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 (PrPSc). Effective therapeutics are currently not available and accurate diagnosis can be challenging.
Brandel, JP +18 more
core +4 more sources
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
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