Sporadic Creutzfeldt-Jakob Disease

The most common type of Creutzfeldt-Jakob Syndrome (85% of cases), where the patient has no known risk factors for the disease, such as inherited PRNP mutations or exposure to animals with BOVINE SPONGIFORM ENCEPHALOPATHY. OMIM: 123400

Also Known As:

Networked: 491 relevant articles (4 outcomes, 37 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Prion Diseases (Transmissible Spongiform Encephalopathies)
2.	Scrapie
3.	Acquired CJD
4.	Kuru
5.	Progressive Supranuclear Palsy (Steele Richardson Olszewski Syndrome)

Experts

1.	Zerr, Inga: 44 articles (09/2022 - 01/2004)
2.	Kitamoto, Tetsuyuki: 28 articles (12/2021 - 12/2003)
3.	Ironside, James W: 25 articles (06/2022 - 12/2003)
4.	Zanusso, Gianluigi: 23 articles (06/2022 - 01/2002)
5.	Llorens, Franc: 21 articles (09/2022 - 01/2014)
6.	Pocchiari, Maurizio: 21 articles (01/2022 - 01/2002)
7.	Schmitz, Matthias: 20 articles (09/2022 - 06/2010)
8.	Parchi, Piero: 18 articles (02/2022 - 04/2003)
9.	Iwasaki, Yasushi: 18 articles (12/2021 - 06/2005)
10.	Mead, Simon: 18 articles (01/2021 - 09/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Sporadic Creutzfeldt-Jakob Disease:

1.	PrionsIBA 11/22/2023 - "Characterisation and prion transmission study in mice with genetic reduction of sporadic Creutzfeldt-Jakob disease risk gene Stx6." 01/01/2022 - "Five hundred and thirty-seven patients were visited by mobile teams of doctors and nurses from the National Health Service National Prion Clinic within 5 days of notification of a suspected diagnosis of sporadic Creutzfeldt-Jakob disease, enrolled to the study between October 2008 and March 2020, and followed up until November 2020. " 11/20/2018 - "This study probes the occurrence, level, and distribution of prions in the eyes of patients with sporadic Creutzfeldt-Jakob disease (sCJD). " 10/01/2016 - "Compared with other bioassays, this assay is extremely sensitive and was used in the present study to determine prion distribution in sporadic Creutzfeldt-Jakob disease patients at autopsy. " 10/10/2023 - "Currently, it is unknown whether infectious prions are present in peripheral tissues and biological fluids of patients affected by sporadic Creutzfeldt-Jakob disease (sCJD), the most common prion disorder in humans. "
2.	Biomarkers (Surrogate Marker)IBA 08/01/2019 - "Fluid biomarkers that can predict survival time in sporadic Creutzfeldt-Jakob disease (sCJD) will be critical for clinical care and for treatment trials. " 01/01/2023 - "Diagnostic performance of CSF biomarkers in a well-characterized Australian cohort of sporadic Creutzfeldt-Jakob disease." 10/18/2022 - "Currently, no established biomarkers exist for presymptomatic sporadic Creutzfeldt-Jakob disease (sCJD). " 09/01/2022 - "Previous observations indicated neurofilament light chain (NfL) as a potential blood-based biomarker for sporadic Creutzfeldt-Jakob disease (sCJD). " 09/01/2022 - "Significance of Cortical Ribboning as a Biomarker in the Prodromal Phase of Sporadic Creutzfeldt-Jakob Disease."
3.	Amyloid (Amyloid Fibrils)IBA 09/01/2015 - "To explore potential mechanistic links between PrionD and AD, we exposed human brain aggregates (BrnAggs) to a brain homogenate from a patient with sporadic Creutzfeldt-Jakob disease and found that neurons in human BrnAggs produced many β-amyloid (Aβ; Aβ42) inclusions, whereas uninfected control-exposed human BrnAggs did not. " 05/01/2005 - "The PK-resistant fragments of the amyloid form are similar to those observed upon PK digestion of a minor subpopulation of PrP(Sc) recently identified in patients with sporadic Creutzfeldt-Jakob disease (CJD). " 01/01/2023 - "The presence of amyloid kuru plaques is a pathological hallmark of sporadic Creutzfeldt-Jakob disease (sCJD) of the MV2K subtype. " 04/01/2013 - "Therefore, ISF blockage is unlikely to be a problem in non-amyloid human prion diseases such as sporadic Creutzfeldt-Jakob disease. " 04/01/2013 - "C57BL/10 mice expressing wild-type anchored PrP, which develop non-amyloid PrPres similar to humans with sporadic Creutzfeldt-Jakob disease, were compared with Tg44+/+ mice (transgenic mice secreting anchorless PrP) expressing anchorless PrP, which develop amyloid PrPres similar to certain human familial prion diseases. "
4.	Prion ProteinsIBA 10/01/2016 - "Prion protein gene sequence, molecular, and neuropathological studies confirmed the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 subtype. " 08/01/2011 - "In this study, we investigated the relationships between neuronal loss and PrP(Sc) deposition in the cerebellum from cases of the six subtypes of sporadic Creutzfeldt-Jakob disease (sCJD; n=100) that can be determined according to the M129V polymorphism of the human prion protein gene (PRNP) and PrP(Sc) molecular types. " 08/01/1996 - "Prion protein genotype and pathological phenotype studies in sporadic Creutzfeldt-Jakob disease." 10/02/2023 - "Elevated E200K Somatic Mutation of the Prion Protein Gene (PRNP) in the Brain Tissues of Patients with Sporadic Creutzfeldt-Jakob Disease (CJD)." 08/02/2023 - "Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal neurodegenerative condition caused by prion proteins. "
5.	Codon (Codons)IBA 07/01/2023 - "Correction: A novel subtype of sporadic Creutzfeldt-Jakob disease with PRNP codon 129MM genotype and PrP plaques." 03/01/2023 - "The polymorphic heterozygosity of PRNP at codon 129 or 219 prevents the onset of sporadic Creutzfeldt-Jakob disease (sCJD). " 01/01/2023 - "A novel subtype of sporadic Creutzfeldt-Jakob disease with PRNP codon 129MM genotype and PrP plaques." 01/01/2021 - "Two distinct conformers of PrPD type 1 of sporadic Creutzfeldt-Jakob disease with codon 129VV genotype faithfully propagate in vivo." 04/01/2015 - "For example, while the most common sporadic Creutzfeldt-Jakob disease (CJD) subtype, sporadic CJD-MM1 (M1 strain), induces a single phenotype after experimental transmission regardless of the codon 129 genotype of the recipient animal, the phenotype elicited by sporadic CJD-VV2 (V2 strain), the second most common subtype, varies according to the host codon 129 genotype. "
6.	Proteins (Proteins, Gene)FDA Link 02/01/2010 - "In the present study, we explored the molecular and biological involvement of PAD2 and the pathogenesis of citrullinated proteins in frontal cortex of patients with sporadic Creutzfeldt-Jakob disease (sCJD). " 02/17/2006 - "In this study, we have compared the pathological profiles and PrP(Sc) characteristics in brains of CWD-affected elk and deer with those in subjects with sporadic Creutzfeldt-Jakob disease (CJD), as well as CJD-affected subjects who might have been exposed to CWD, using histopathology, immunohistochemistry, immunoblotting, conformation stability assay, and N-terminal protein sequencing. " 01/01/2021 - "Development and clinical application of disease-specific protein aggregation and amplification assays, such as real-time quaking induced conversion (RT-QuIC), have constituted major breakthroughs for the confident pre-mortem diagnosis of sporadic Creutzfeldt-Jakob disease. " 01/01/2016 - "Neuron loss, synaptic decline, and spongiform change are the hallmarks of sporadic Creutzfeldt-Jakob disease (sCJD), and may be related to deficiencies in mitochondria, energy metabolism, and protein synthesis. " 06/10/2014 - "Interpretation of cerebrospinal fluid protein tests in the diagnosis of sporadic Creutzfeldt-Jakob disease: an evidence-based approach."
7.	Valine (L-Valine)FDA Link 08/01/2001 - "Sporadic Creutzfeldt-Jakob disease in a young Dutch valine homozygote: atypical molecular phenotype." 12/01/2001 - "Thus, it might be suggested that this case showed simultaneously the features of both sporadic Creutzfeldt-Jakob disease (CJD) with codon-129 valine and Parkinson's disease. " 01/01/2018 - "Our findings further indicate that the valine-to-isoleucine substitution is not a polymorphism, but rather an authentic pathogenic mutation associated with specific biochemical characteristics that differ from those observed in sporadic Creutzfeldt-Jakob disease." 02/01/2003 - "We describe a 67-year-old Japanese man with probable sporadic Creutzfeldt-Jakob disease (CJD) who had valine homozygosity at codon 129, a rarity in the Japanese. " 02/01/2003 - "Probable sporadic Creutzfeldt-Jakob disease with valine homozygosity at codon 129 and bilateral middle cerebellar peduncle lesions."
8.	Methionine (L-Methionine)FDA Link 12/01/2021 - "Methionine/methionine type 1 (MM1-type) sporadic Creutzfeldt-Jakob disease (sCJD), known as the 'classic type,' shows typical clinicopathological sCJD findings. " 12/01/2020 - "Periodic sharp wave complexes (PSWCs), identified using electroencephalography, are observed in less than half of patients with the methionine homozygosity type 2 cortical (MM2c) form of sporadic Creutzfeldt-Jakob disease (sCJD), and only at a later stage of the disease. " 10/01/2010 - "Among atypical sporadic Creutzfeldt-Jakob disease cases, the MM2 type appeared most common, probably related to the very high proportion of methionine allele in the Japanese population. " 06/01/2006 - "Methionine-methionine (MM) homozygosity at codon 129 is a risk factor for sporadic Creutzfeldt-Jakob disease (CJD). " 10/05/2001 - "However, two distinct disease phenotypes, identified as sporadic Creutzfeldt-Jakob disease (M/M2 sCJD) and sporadic fatal insomnia (sFI), share methionine homozygosity at codon 129 and PrP(Sc) type 2. One-dimensional gel electrophoresis and immunoblotting reveal no difference between the M/M2 sCJD and sFI species of PrP(Sc) in gel mobility and glycoform ratio. "
9.	Peptide Hydrolases (Proteases)FDA Link 03/01/2011 - "In this study, we investigated PrP(Sc) in different brain regions in variant and sporadic Creutzfeldt-Jakob disease (CJD) by using CDI, and directly compared the results with those obtained using the more commonly employed protease digestion and Western blotting. " 12/01/2014 - "Here, we show that protease-sensitive abnormal PrP aggregates derived from patients with sporadic Creutzfeldt-Jakob disease are taken up and degraded by immortalized human astrocytes similarly to abnormal PrP aggregates that are resistant to proteases. " 12/01/2014 - "In sporadic Creutzfeldt-Jakob disease, disease-associated PrP(Sc) is present not only as an aggregated, protease-resistant form but also as an aggregated protease-sensitive form (sPrP(Sc)). " 11/01/1998 - "Fourth, protease-resistant PrPSc cores in sporadic Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker F198S disease brains all have an intact C1 cleavage site (Met111-His112), which precludes disruption of a domain associated with toxicity and fibrillogenesis. " 10/21/2014 - "Variably protease sensitive prionopathy (VPSPr) is a recently described, sporadic human prion disease that is pathologically and biochemically distinct from the currently recognised sporadic Creutzfeldt-Jakob disease (sCJD) subtypes. "
10.	CytokinesIBA 01/01/2014 - "The present study identifies deregulated cytokines and mediators of the immune response in the frontal cortex and cerebellum of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 and VV2 subtypes compared to age-matched controls. " 02/04/2016 - "Gene expression studies of cytokines and mediators of the immune response have been made in post-mortem human brain samples in AD, sPD, sporadic Creutzfeldt-Jakob disease (sCJD) subtypes MM1 and VV2, Pick's disease (PiD), progressive supranuclear palsy (PSP) and frontotemporal lobar degeneration linked to mutation P301L in MAPT Frontotemporal lobar degeneration-tau (FTLD-tau). "

Therapies and Procedures

1.	Operative Surgical Procedures 10/01/2017 - "Risk of transmission of sporadic Creutzfeldt-Jakob disease by surgical procedures: systematic reviews and quality of evidence." 01/01/2006 - "In this preparatory phase of a case-control study, we propose and evaluate a new tool for classifying surgical procedures (SPs) in categories useful for epidemiologic research on surgical transmission of sporadic Creutzfeldt-Jakob disease (sCJD). " 01/01/2006 - "Classification of surgical procedures for epidemiologic assessment of sporadic Creutzfeldt-Jakob disease transmission by surgery." 02/01/2009 - "To elucidate the association between medical procedures and sporadic Creutzfeldt-Jakob disease (sCJD), we analyzed medical procedures (any surgical procedure, neurosurgery, ophthalmic surgery, and blood transfusion) for patients registered by the CJD Surveillance Committee in Japan during 1999-2008. " 01/01/2008 - "The extra neural tissue distribution of variant and sporadic Creutzfeldt-Jakob disease has increased concern regarding the risk of prion disease transmission via general surgical procedures and highlighted the need for decontamination procedures that can be incorporated into routine processing. "
2.	Therapeutics 01/01/2018 - "A blood-based biomarker of neuronal damage in sporadic Creutzfeldt-Jakob disease (sCJD) will be extremely valuable for both clinical practice and research aiming to develop effective therapies. " 09/08/2014 - "Symptomatic aggravation after corticosteroid pulse therapy in definite sporadic Creutzfeldt-Jakob disease with the feature of Hashimoto's encephalopathy." 03/01/2010 - "We describe a patient in whom sporadic Creutzfeldt-Jakob disease (sCJD) occurred one year after the onset of etanercept therapy for rheumatoid arthritis (RA). " 01/01/2004 - "We have compared this with the distribution and biochemical forms found in all of the major subtypes of sporadic Creutzfeldt-Jakob disease and in a case of iatrogenic Creutzfeldt-Jakob disease associated with growth hormone therapy. " 10/01/2003 - "The relationships between the degree of cortical prion protein (PrP) deposition, tissue vacuolation and astrocytosis were studied in the frontal cortex of 27 cases of human spongiform encephalopathy, encompassing 13 cases of sporadic Creutzfeldt-Jakob disease (sCJD), four cases of familial CJD (fCJD) (one owing to E200K mutation, one owing to 144 bp insertion, one owing to P102L mutation and one owing to A117V mutation), five cases of iatrogenic CJD (iCJD) owing to growth hormone therapy and five cases of variant CJD (vCJD). "
3.	Blood Transfusion (Blood Transfusions) 08/01/2011 - "Sporadic Creutzfeldt-Jakob disease and risk of blood transfusion in the United Kingdom." 07/01/2011 - "Transmission of sporadic Creutzfeldt-Jakob disease by blood transfusion: risk factor or possible biases." 02/01/2009 - "To elucidate the association between medical procedures and sporadic Creutzfeldt-Jakob disease (sCJD), we analyzed medical procedures (any surgical procedure, neurosurgery, ophthalmic surgery, and blood transfusion) for patients registered by the CJD Surveillance Committee in Japan during 1999-2008. "
4.	Transplantation 09/01/2006 - "Transplantation of ocular tissue from a donor with sporadic Creutzfeldt-Jakob disease." 11/20/2018 - "Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common prion disease in humans and has been iatrogenically transmitted through corneal graft transplantation. "
5.	Surgical Instruments (Clip) 01/01/2016 - "We build a few population-based and experimentally-testable hypotheses focusing on: (1) non-disposable surgical instruments for sporadic Creutzfeldt-Jakob disease (sCJD) and other rapid progressive neurodegenerative dementia (sRPNDd), multiple system atrophy (MSA), and motor neuron disease (MND); and (2) specific bacterial infections such as B. " 12/01/2009 - "In aetiological viewpoint, human prion diseases are classified into 1) sporadic Creutzfeldt-Jakob disease (CJD) which comprises 80-90% of the total population of human prion disaeses, 2) inherited forms, and 3) acquired types by prion-contaminated surgical instruments, biopharmaceuticals or foodstuffs. "