Acquired CJD

Spontaneously-occuring (sporadic) cases of Creutzfeldt-Jakob Disease or cases acquired through innoculation during medical procedures or through consumption of BSE-containing food products.

Also Known As:

Networked: 243 relevant articles (9 outcomes, 38 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Sporadic Creutzfeldt-Jakob Disease
2.	Kuru
3.	Scrapie
4.	Bovine Spongiform Encephalopathy (Mad Cow Disease)
5.	Creutzfeldt-Jakob Syndrome (Creutzfeldt-Jakob Disease)

Experts

1.	Kitamoto, Tetsuyuki: 18 articles (01/2022 - 12/2003)
2.	Parchi, Piero: 15 articles (01/2020 - 01/2003)
3.	Zerr, Inga: 13 articles (12/2020 - 01/2002)
4.	Gambetti, Pierluigi: 12 articles (11/2019 - 01/2003)
5.	Kobayashi, Atsushi: 10 articles (10/2021 - 02/2009)
6.	Pocchiari, Maurizio: 10 articles (12/2020 - 01/2007)
7.	Mohri, Shirou: 9 articles (10/2021 - 12/2003)
8.	Capellari, Sabina: 8 articles (01/2020 - 11/2007)
9.	Ironside, James W: 8 articles (12/2017 - 12/2003)
10.	Collinge, John: 8 articles (11/2017 - 12/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Acquired CJD:

1.	Codon (Codons)IBA 08/01/2010 - "A further genetic polymorphism at codon 219, a common variant in several Asian populations, is considered protective against sporadic CJD. " 10/19/2012 - "This study confirmed the presence of three distinct sporadic CJD molecular subtypes with PrP(C) substrate requirements that reflected their codon 129 associations in vivo. " 12/01/2005 - "Our study showed significant differences in genotype frequency of PRNP at codon 129 (chi 2=8.8998, P=0.0117) or 219 (chi 2=12.6945, P=0.0004) between sporadic CJD and normal controls. " 01/01/2005 - "In contrast to results in the German population, our study did not show a significant difference in PRND genotype or allele frequency at codon 174 between sporadic CJD and normal controls. " 01/01/2022 - "The recent diagnosis of vCJD in a patient heterozygous (MV) at codon 129 reignited concerns regarding a second wave of vCJD cases, with the possibility of phenotypic divergence from MM vCJD and greater overlap with sporadic CJD (sCJD) molecular subtypes. "
2.	PrionsIBA 10/15/1999 - "Our study suggests that human sporadic CJD-related prions display a significant heterogeneity." 01/01/2023 - "Our data show that both eLpH and Wex-cide 128 removed 4.0-5.5 logs of prion infectivity from 22L, CWD and 263K prion homogenates, but only about 1.25-1.50 logs of prion infectivity from human sporadic CJD. " 10/07/2021 - "While in all cases the unglycosylated fragment in Western blot analysis shared the same size with sporadic CJD prion type 2, the reticular/synaptic deposition pattern of the prion aggregates resembled the ones found in sporadic CJD type 1 (CJD types according to the Parchi classification from 1999). " 01/01/2020 - "However, this assay is currently unable to detect prions associated with the sporadic CJD (sCJD) forms, which are more frequent than vCJD. " 01/01/2020 - "Preliminary data based on a limited number of cases suggest that prion-seeding activity in the skin varies according to the prion strain, being higher in sporadic CJD subtypes linked to the V2 strain (VV2 and MV2K) than in typical CJDMM1."
3.	14-3-3 Proteins (14-3-3 Protein)IBA 06/01/2003 - "Previous studies have suggested that, in the proper clinical context, the 14-3-3 protein in cerebrospinal fluid is a reliable marker for sporadic CJD. " 01/01/2018 - "Observation of intense signal changes in cortical regions (cortical ribboning) in diffusion weighted imaging (DWI) MRI scanning and positive protein 14-3-3 in cerebrospinal fluid (CSF) proposed the diagnosis of sporadic CJD. " 03/01/2015 - "Next, CSF t-PrP concentration was measured in a cohort of 104 patients including 55 patients with probable AD, 26 with probable sporadic CJD, and 23 control patients for whom 14-3-3 protein, total tau, phosphorylated tau 181 (P-tau181), and Aβ1-42 were available. " 06/01/2012 - "Protein 14-3-3 in the cerebrospinal fluid was examined in a single patient and was positive, allowing the clinical classification as probable sporadic CJD. " 11/01/2010 - "14-3-3 protein detection and sporadic CJD: the status quo serves well while awaiting progress."
4.	Biomarkers (Surrogate Marker)IBA 12/01/2020 - "At this point, we sought to characterise the biomarker profile of iCJD and compare it to that of sporadic CJD (sCJD) for determining the value of available diagnostic tools in promptly recognising iCJD cases. " 09/04/2012 - "The putative sporadic CJD biomarkers were then validated independently by immunoturbidimetry. " 07/29/2010 - "A disease-specific tau band emerged in the CSF samples from probable sporadic CJD, which may supply a new biomarker for screening sporadic CJD." 08/01/2015 - "The present study examined prion biomarker levels in the brain and CSF of sporadic CJD (sCJD) cases and their correlation with neuropathological lesion profiles. " 09/04/2012 - "The possible value of this set of proteins as biomarkers in the diagnosis of sporadic CJD or for blood/tissue donor screening remains to be further explored and validated in larger studies."
5.	Methionine (L-Methionine)FDA Link 10/01/2000 - "In conclusion, these cases exhibited a fairly uniform phenotype, which is relatively distinct from sporadic CJD in methionine homozygotes, and thus diagnosis may be difficult using existing clinical criteria." 01/01/2022 - "For patients with methionine homozygosity, potential risk for occurring prion diseases: adjusted OR (95% CI) was 2.21 (1.46 to 3.34) in sporadic CJD, 0.47 (0.32 to 0.68) in genetic CJD and 0.3 (0.17 to 0.55) in GSS. " 02/01/2009 - "To determine in the cerebellum in variant Creutzfeldt-Jakob disease (vCJD): (i) whether the pathology affected all laminae; (ii) the spatial topography of the pathology along the folia; (iii) spatial correlations between the pathological changes; and (iv) whether the pathology was similar to that of the common methionine/methionine Type 1 subtype of sporadic CJD. " 01/01/2022 - "Methionine homozygosity at codon 129 was predisposing to sporadic CJD, but protective against genetic CJD and GSS, after adjustment for codon 219 polymorphism effect. " 01/01/2016 - "These findings suggest that our cell-PMCA is a useful tool for easily and rapidly identifying acquired CJD associated with the transmission of the V2 CJD strain to codon 129 methionine homozygotes, based on the preference for the 129 V substrate and the type of the amplified products."
6.	Proteins (Proteins, Gene)FDA Link 02/01/2014 - "Global protein differential expression profiling of cerebrospinal fluid samples pooled from Chinese sporadic CJD and non-CJD patients." 09/04/2012 - "Increased levels of acute-phase inflammatory proteins in plasma of patients with sporadic CJD." 02/01/2007 - "Total protein concentrations of >0.9 g/l were found in five of 438 patients with sporadic CJD, although none had a concentration of >1 g/l. " 12/01/2006 - "The present findings demonstrate oxidative, glycoxidative, lipoxidative and nitrative protein damage, accompanied by increased oxidative responses, in the cerebral cortex in sporadic CJD. " 08/01/2002 - "In 1986 two proteins were detected in the cerebrospinal fluid (CSF) of patients with sporadic CJD. "
7.	tau Proteins (tau Protein)IBA 03/01/2003 - "However, false negative results in autopsy-proven, sporadic CJD cases, as well as false positive results in several other disorders including AD and FTD showing high CSF tau protein levels, limit the potential of this marker. " 04/01/2021 - "In the present study, we did a meta-analysis on the liability of RT-QuIC method in the diagnosis of sporadic CJD, in comparison to 14-3-3 and Tau protein. " 10/01/2010 - "This study investigated the diagnostic sensitivity and specificity of the microtubule-association protein tau in cerebrospinal fluid (CSF) from Chinese patients with sporadic CJD. " 01/01/2006 - "CSF tau protein and tau-pT181 were measured in 50 patients with sporadic CJD (sCJD), 51 patients with vCJD, 46 sCJD controls, and 37 vCJD controls, using Innotest hTau and Innotest P-Thr181, Innogenetics. " 09/01/2001 - "Highly increased CSF tau protein and decreased beta-amyloid (1-42) in sporadic CJD: a discrimination from Alzheimer's disease?"
8.	SynucleinsIBA 01/01/2022 - "Several studies now suggest that PD patients can be subcategorized based on where in the body misfolded α-synuclein originates, either the brain or the gut, similar to patients developing sporadic CJD or vCJD. " 12/01/2020 - "Provided that increased APs evolvability might be beneficial for acquired CJD in young adults, a dose-reduction of α-synuclein, a natural inhibitor of αS aggregation, might be therapeutically effective in upregulating APs evolvability. "
9.	PrPSc ProteinsIBA 07/01/2006 - "In sporadic CJD, PrP(CJD) accumulated only in the cerebellar molecular layer in the area of the synapses formed by climbing fibers with the dendrites of Purkinje cells. " 01/01/2002 - "PrP(RES) in vCJD brain tissue showed a uniform glycotype pattern distinct from sporadic CJD. " 08/01/2004 - "Autopsy and biopsy brain material from sporadic CJD cases also showed an increased sensitivity of PrP(res) detection with the PET blot, confirming its value as an important diagnostic and research tool in human prion diseases." 01/01/2016 - "Recently, novel ultrasensitive seeding assays, based on the amplified detection of PrP(CJD), have improved the diagnostic process; for example, real-time quaking-induced conversion (RT-QuIC) is a sensitive method to detect prion-seeding activity in brain homogenate from humans with any subtype of sporadic CJD. " 01/01/2002 - "Comparison of the PrP(res) positivity in the cerebellum of familial and sporadic CJD using specific polyclonal and monoclonal antibodies (MAbs) to PrP showed less conspicuous immune reaction in CJD(E200K) cases. "
10.	Amyloid (Amyloid Fibrils)IBA 01/01/2012 - "Cortical areas of variant Creutzfeldt-Jakob disease (CJD; n = 3), young sporadic CJD (n = 4), different Gerstmann-Sträussler-Scheinker's disease patients (n = 5) and AD cases (n = 5) were examined using immunohistochemistry and specific stainings for amyloid. " 07/01/2004 - "Many forms of human prion disease including variant Creutzfeldt-Jakob disease (vCJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome, and 10% of sporadic CJD cases are associated with amyloid deposition. " 10/01/1995 - "Using an improved extraction method and Western blot detection, the disease-specific amyloid was found in various parts of the central nervous system of hamsters orally infected with scrapie, of squirrel monkeys orally infected with kuru, sporadic Creutzfeldt-Jakob disease (CJD) and scrapie, of human patients with sporadic CJD, of a sheep with natural scrapie and of a cow with bovine spongiform encephalopathy (BSE). " 04/01/2021 - "More recently, a new method for the pre-mortem diagnosis of sporadic CJD has been developed, based on the ability of PrPsc to induce the polymerization of protease-sensitive recombinant PrP (PrPsen) into amyloid fibrils, and is known as Real-Time Quaking- Induced Conversion (RT-QuIC) assay allows the detection of > 1 fg of PrPsc in diluted CJD brain homogenate and a variety of biological tissues and fluids. " 01/01/2018 - " investigate this phenomenon further, a cohort of 27 iCJD cases - 21 with adequate number of histopathological sections - originating from Australia, France, Italy, and the Unites States, were examined by immunohistochemistry, amyloid staining, and Western blot analysis of the scrapie prion protein (PrPSc), and compared with age-group matched cases of sporadic CJD (sCJD), Alzheimer disease (AD) or free of neurodegenerative diseases (non-ND).Cases of iCJD and sCJD shared similar profiles of proteinase K-resistant PrPSc with the exception of iCJD harboring the "MMi" phenotype. "

Therapies and Procedures

1.	Drug Tapering 12/01/2020 - "Provided that increased APs evolvability might be beneficial for acquired CJD in young adults, a dose-reduction of α-synuclein, a natural inhibitor of αS aggregation, might be therapeutically effective in upregulating APs evolvability. "
2.	Blood Transfusion (Blood Transfusions) 01/01/2020 - "This study aims to corroborate the scarce data that suggest that sporadic CJD (sCJD) is not transmitted via blood transfusion. " 01/01/2012 - "For sporadic CJD (sCJD), a history of surgery or blood transfusion was associated with risk in some, but not all, recent studies at a ≥10 year lag time, when controls were longitudinally sampled. " 07/01/2011 - "CJD with a definite or probable diagnosis (sporadic CJD, n = 741; genetic CJD, n = 175) and no-CJD patients with definite alternative diagnosis (n = 482) with available blood transfusion history were included in the study. " 06/01/2003 - "However, in man, epidemiological case control, lookback and surveillance studies have failed to demonstrate any evidence of sporadic CJD transmission by blood transfusion. " 02/07/2004 - "Epidemiological evidence does not suggest that sporadic CJD is transmitted from person to person via blood transfusion, but this evidence may not apply to vCJD. "
3.	Operative Surgical Procedures 08/27/2002 - "A history of surgery was significantly associated with the risk of sporadic CJD (odds ratio [OR]: 1.8; 95% CI: 1.2 to 2.6), which was not dependent on the number of surgical procedures, and was stronger in females (OR: 2.5; 95% CI: 1.5 to 4.0). " 02/27/1999 - "Surgical procedures were significantly associated with the development of sporadic CJD. "
4.	Corneal Transplantation (Keratoplasty) 08/01/2008 - "Because of the many corneal transplantations performed each year in the United States, occasional cases of sporadic CJD in this population are expected." 08/01/2008 - "Using corneal transplantation and CJD death data from 1990 through 2006, statistical analyses suggest that a case of coincidental sporadic CJD will occur among the population of corneal transplant recipients approximately every 1.5 years. "
5.	Transplantation 02/01/2008 - "No differences between our dura-CJD cases and typical cases of sporadic CJD were found with respect to clinicopathologic findings, except history of dura mater transplantation. "