Creutzfeldt-Jakob Disease With Florid-Type Plaques After Cadaveric Dura Mater Grafting

Shimizu, Saori; Hoshi, K.; Muramoto, Tamaki; Homma, Mari; Ironside, James W.; Kuzuhara, Shigeki; Satô, Takeshi; Yamamoto, Toshiyuki; Kitamoto, Tetsuyuki

doi:10.1001/archneur.56.3.357

Cited by 79 publications

(80 citation statements)

References 22 publications

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“…Assuming there is no difference between the treatment results, use of the infection-free hemostatic material that does not include animal-derived collagen or human blood components would be preferable. Biological viral infections such as iatrogenic hepatitis from infected blood products 16) and iatrogenic Creutzfeldt-Jakob disease (CJD) from artificial dura mater or corneal transplant 17,18) have occurred in Japan and have become social problems. In the case of products derived from biological sources, the presence of unknown viruses cannot be completely ruled out.…”

Section: Discussionmentioning

confidence: 99%

Novel Infectious Agent-Free Hemostatic Material (TDM-621) in Cardiovascular Surgery

Masuhara

Fujii

Watanabe

et al. 2012

Ann Thorac Cardiovasc Surg

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Subjects: Currently, hemostatic materials made from human blood components and animalderived collagen is used for controlling operative hemorrhage in the cardiovascular surgery field. In this study, we focused on an entirely synthetic self-assembling peptide (development code: TDM-621) that gels when in contact with blood or other bodily fluids and stops bleeding upon contact with a wound site. We investigated its usefulness as a hemostatic material in animal and clinical studies. Methods: Before we began the clinical study, we demonstrated the hemostasis efficacy and safety of TDM-621 in animal experimental models. Twenty-five patients (22 men, 3 women) were enrolled in the clinical study, and the following procedures were performed: 1) coronary artery bypass graft (CABG) (n = 9), 2) abdominal aortic graft replacement (n = 4), and 3) peripheral artery bypass (n = 12). The TDM-621 material was applied to a total of 33 vascular anastomotic graft sites (some patients received material at more than one site). Both hemostatic efficacy and safety were examined. Results: A total of 33 anastomotic graft sites in 25 patients were evaluated, and the averaged primary and secondary efficacy rate was 94.5%. No postoperative bleeding or adverse events (including serious adverse events) with a causal relationship to treatment were observed. Conclusion: This study indicated that TDM-621 is a more effective and reliable hemostat than commonly-used general hemostatic agents and, therefore, will be very useful in several cardiovascular surgery applications.

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Section: Discussionmentioning

confidence: 99%

Novel Infectious Agent-Free Hemostatic Material (TDM-621) in Cardiovascular Surgery

Masuhara

Fujii

Watanabe

et al. 2012

Ann Thorac Cardiovasc Surg

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“…Given that the PrP res sizes reflect the PrP Sc conformation to some degree, 26 Sc conformation has more influence on the transmission efficiency of prions than the primary structure, being consistent with recently reported experiments. 28,29 In addition to the identical primary structure, the PrP res of the three prions, MM1-sCJD, np-dCJD, and p-dCJD have the identical PrP res size of 21 kd, being practically indistinguishable from one another; 16 despite that, p-dCJD never transmitted to the Ki-ChM mouse, whereas the other two prions transmitted very efficiently. Such heterogeneity in transmission properties among prions with the identical type of PrP res and identical etiology is also observed in other transmission studies; 11,13 this suggests a considerable heterogeneity among the prions categorized into the same group based on the properties of PrP res and etiology.…”

Section: Discussionmentioning

confidence: 99%

“…16 The diagnosis of prion disease was confirmed by identifying characteristic neuropathological findings such as spongiform change, gliosis, and PrP deposition of formalin-fixed brain sections, 4,17 and by identifying PrP res extracted from the brain tissues on Western immunoblots as described. 16 Typing of PrP res was performed based on the classification proposed by Parchi and colleagues: 3,7 for sporadic CJD cases, PrP res of ϳ21 kd was designated as PrP res type 1, and PrP res of ϳ19 kd as PrP res type 2 ( Figure 1A). PrP genotyping of patients was performed by polymerase chain reaction-direct sequencing using genomic DNA purified from brain tissues or peripheral blood leukocytes.…”

Section: Human Prion Diseases Inoculatedmentioning

confidence: 99%

“…PrP genotyping of patients was performed by polymerase chain reaction-direct sequencing using genomic DNA purified from brain tissues or peripheral blood leukocytes. 18 After characterization of the genotype and PrP res typing, the brain tissues from different prion disease cases 16 and two cases of inherited prion diseases [one of GSS with the P102L mutation (GSS102) and one of CJD with the M232R mutation (CJD232)] ( Figure 1A, Table 1). All of the cases of vCJD, dCJD, and inherited prion diseases were homozygous for methionine at codon 129; as for the another polymorphic codon, codon 219, all of the cases collected were homozygous for glutamate, except for GSS102, which had lysine at codon 219 on the allele without P102L mutation.…”

Section: Human Prion Diseases Inoculatedmentioning

confidence: 99%

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Humanized Knock-In Mice Expressing Chimeric Prion Protein Showed Varied Susceptibility to Different Human Prions

Taguchi

Mohri

Ironside

et al. 2003

The American Journal of Pathology

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Mice to which human prions efficiently transmit in short incubation periods are valuable not only as research tools of human prions but also as reliable diagnostic tools. We recently produced a line of knock-in mouse expressing a unique human-mouse chimeric PrP (Ki-ChM mouse), which has mouse-specific residues practically only at the C-terminal part after posttranslational modification, and here we attempted transmission of various human prions to assess the susceptibility profile of the mouse. Susceptibility varied considerably depending on prions inoculated: highly susceptible to MM1 and MV1 types of sporadic Creutzfeldt-Jakob disease (CJD), developing disease within ϳ150 days, familial CJD with M232R mutation, and dura graft-associated CJD (dCJD) without amyloid plaque; less susceptible to MM2-type sporadic CJD and variant CJD, with some mice lacking any sign of transmission; and totally resistant to VV2 type sporadic CJD and dCJD with amyloid plaque. The rather short incubation time achieved by Ki-ChM mice suggests new approaches to produce mice that develop prion disease with very short incubation periods. We compared the characteristic susceptibility profile of Ki-ChM with those of other precedent transgenic mice and discussed , including the prospects in developing genetically engineered mice susceptible to human prions.

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“…The first is a major group represented by a non-plaque-type dCJD (np-dCJD), the second a minor group represented by a plaque-type dCJD (p-dCJD). [5][6][7] np-dCJD shares phenotypic characteristics such as diffuse synaptic-PrP deposition and type 1 PrP Sc with sCJD-MM1 or -MV1, the most common sCJD phenotype (denoted as M1 strain in transmission studies). 4,8 In contrast, p-dCJD is characterized by unusual phenotypic features such as the presence of numerous kuru plaques and a unique PrP Sc type with an electrophoretic mobility of about 20 kDa, which is intermediate in size between types 1 and 2 (type i PrP Sc ).…”

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confidence: 99%

Distinctive properties of plaque-type dura mater graft-associated Creutzfeldt–Jakob disease in cell-protein misfolding cyclic amplification

Takeuchi

Kobayashi

Parchi

et al. 2016

Laboratory Investigation

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There are two distinct subtypes of dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) with methionine homozygosity at codon 129 of the PRNP gene. The majority of cases is represented by a non-plaque-type (np-dCJD) resembling sporadic CJD (sCJD)-MM1 or -MV1, while the minority by a plaque-type (p-dCJD). p-dCJD shows distinctive phenotypic features, namely numerous kuru plaques and an abnormal isoform of prion protein (PrP Sc ) intermediate in size between types 1 and 2. Transmission studies have shown that the unusual phenotypic features of p-dCJD are linked to the V2 prion strain that is associated with sCJD subtypes VV2 or -MV2. In this study, we applied protein misfolding cyclic amplification (PMCA) using recombinant human prion protein as a substrate and demonstrated that p-dCJD prions show amplification features that are distinct from those of np-dCJD. Although no amplification of np-dCJD prions was observed with either 129 M or 129 V substrate, p-dCJD prions were drastically amplified with the 129 V substrates, despite the PRNP codon 129 incompatibility between seed and substrate. Moreover, by using a type 2 PrP Sc -specific antibody not recognizing PrP Sc in p-dCJD, we found that type 2 products are generated de novo from p-dCJD prions during PMCA with the 129 V substrates. 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.

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Creutzfeldt-Jakob Disease With Florid-Type Plaques After Cadaveric Dura Mater Grafting

Abstract: These 2 cases are clinicopathologically distinct from typical dura-associated cases of CJD. They may be a subtype with florid-type plaques in dura-associated CJD.

Cited by 79 publications

References 22 publications

Novel Infectious Agent-Free Hemostatic Material (TDM-621) in Cardiovascular Surgery

Novel Infectious Agent-Free Hemostatic Material (TDM-621) in Cardiovascular Surgery

Humanized Knock-In Mice Expressing Chimeric Prion Protein Showed Varied Susceptibility to Different Human Prions

Distinctive properties of plaque-type dura mater graft-associated Creutzfeldt–Jakob disease in cell-protein misfolding cyclic amplification

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