Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999–2008

Hamaguchi, Tetsuya; Noguchi‐Shinohara, Moeko; Nozaki, Ichiro; Nakamura, Yosikazu; Satô, Takeshi; Kitamoto, Tetsuyuki; Mizusawa, Hidehiro; Yamada, Masahito

doi:10.3201/eid1502.080749

Cited by 27 publications

(62 citation statements)

References 20 publications

(33 reference statements)

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“…Furthermore, the other patient in the present study (case 2), who also showed distinctive phenotype and transmission properties similar to those of plaque-type dCJD, had a medical history of neurosurgery without dura mater grafting, suggesting that she might have also been infected through cross-contamination from operative instruments. During the period 1999 to 2008, the Japanese CJD Surveillance registry listed 6 of 760 sCJD patients who had undergone neurosurgery after the onset but before the diagnosis of sCJD (27). Although none of the individuals exposed to possibly contaminated instruments has developed CJD (as of September 2014), the ensemble of these observations suggests that the potential risk of iatrogenic transmission via neurosurgical procedures may be greater than is presently appreciated.…”

Section: Discussionmentioning

confidence: 81%

Transmission Properties of Atypical Creutzfeldt-Jakob Disease: a Clue to Disease Etiology?

Kobayashi

Parchi

Yamada

et al. 2015

J Virol

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The genotype at polymorphic codon 129 of the PRNP gene has a profound influence on both phenotypic expression and prion strain susceptibility in humans. 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. In particular, the propagation of the V2 strain in codon 129 methionine homozygotes has been linked only to acquired forms of CJD such as plaque-type dura mater graft-associated CJD (dCJD), a subgroup of iatrogenic CJD with distinctive phenotypic features, but has never been observed in sporadic CJD cases. In the present report, we describe atypical CJD cases carrying codon 129 methionine homozygosity, in a neurosurgeon and in a patient with a medical history of neurosurgery without dural grafting, showing the distinctive phenotypic features and transmission properties of plaque-type dCJD. These findings raise the possibility that the two cases, previously thought to represent sporadic CJD, might actually represent acquired CJD caused by infection with the V2 strain. Thus, careful analyses of phenotypic features and transmission properties in atypical cases may be useful to distinguish acquired from sporadic cases of CJD. IMPORTANCESusceptibility to and phenotypic expression of Creutzfeldt-Jakob disease (CJD) depend on both the prion strain and genotype at polymorphic codon 129 of the PRNP gene. For example, propagation of the second most common sporadic CJD strain (V2 strain) into codon 129 methionine homozygotes has been linked to plaque-type dura mater graft-associated CJD (dCJD), a subgroup of iatrogenic CJD with distinctive phenotypic features, but has never been observed in sporadic CJD. In the present report, we describe atypical CJD cases in a neurosurgeon and in a patient with a medical history of neurosurgery without dural grafting, showing the distinctive phenotypic features and transmission properties of plaque-type dCJD. These findings raise the possibility that the two cases, previously considered to represent sporadic CJD, might actually represent acquired CJD caused by infection with the V2 strain.

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Section: Discussionmentioning

confidence: 81%

Transmission Properties of Atypical Creutzfeldt-Jakob Disease: a Clue to Disease Etiology?

Kobayashi

Parchi

Yamada

et al. 2015

J Virol

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“…The search on reports up to 2011 provided 20 full-text documents on 18 case-control studies on CJD where BT or medical procedures were mentioned as potential exposures in the Methods or Results, either as the primary subject of study or as one of a number of potential risk factors, e.g. genetic, occupational or dietary [4,5,9,10,13,14,15,16,17,26,27,28,29,30,31,32,33,34,35,36] (see fig. 1 for details of the selection flow).…”

Section: Methodsmentioning

confidence: 99%

“…In order to improve completeness, 5 research reports, 3 of which were published before 1990, and 2 recent papers on BT and surgical history were added [13,14,15,16,17]. Thirty-three documents pertaining to case-control studies and several studied cohorts [4,5,6,7,8,9,10,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38] were examined in full text. Thirteen were excluded from the systematic review since: (a) they focused on biographic residential space-time relationships [19]; (b) they were methodological [6,7,8,20]; (c) they focused on the follow-up of national single cohorts of persons exposed to treatment with human pituitary growth hormones in the UK, The Netherlands and the USA [18,21,22,23,24], or with BT or plasma products without data on nonexposed persons [37,38], or (d) they related to human dura mater graft recipients [25].…”

Section: Methodsmentioning

confidence: 99%

Sensitivity to Biases of Case-Control Studies on Medical Procedures, Particularly Surgery and Blood Transfusion, and Risk of Creutzfeldt-Jakob Disease

Cuesta¹,

Ruiz-Tovar²,

Ward

et al. 2012

Neuroepidemiology

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Background: Evidence of risk of Creutzfeldt-Jakob disease (CJD) associated with medical procedures, including surgery and blood transfusion, is limited by susceptibility to bias in epidemiological studies. Methods: Sensitivity to bias was explored using a central-birth-cohort model using data from 18 case-control studies obtained after a review of 494 reports on medical procedures and risk of CJD, systematic for the period January 1, 1989 to December 31, 2011. Results: The validity of the findings in these studies may have been undermined by: recall; control selection; exposure assessment in life-time periods of different duration, out of time-at-risk of effect, or asymmetry in case/control data; and confounding by concomitant blood transfusion at the time of surgery. 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. Space-time aggregation of surgical events was not seen. Surgery at early clinical onset might be overrepresented among cases. Neither surgical history nor blood transfusion unlabelled for donor status, dental treatments or endoscopic examinations were linked to variant CJD (vCJD). Conclusions: These results indicate the need for further research. Common challenges within these studies include access to and content of past medical/dental treatment records for diseases with long incubation periods.

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“…Medical procedures such as neurosurgery and ophthalmic surgery before the diagnosis of CJD may be associated with risk of secondary transmission of CJD through contaminated instruments. Our recent study revealed that 0.8% of patients with CJD underwent neurosurgery after the onset, but before the diagnosis of CJD, and Noguchi-Shinohara et al 14 1.8% of patients with sporadic CJD ophthalmic surgery [21]. Measurement of serum-tau before neurosurgery and ophthalmic surgery in patients with dementia may be a simple and useful tool for the screening of CJD.…”

Section: Discussionmentioning

confidence: 99%

Serum tau protein as a marker for the diagnosis of Creutzfeldt-Jakob disease

et al. 2011

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Total tau protein (t-tau) levels in cerebrospinal fluid (CSF) (CSF-tau) are markedly elevated in patients with Creutzfeldt-Jakob disease (CJD). Some CSF-tau may leak into the blood. We evaluated t-tau levels in serum (serum-tau) as a possible marker for the differential diagnosis of CJD from Alzheimer's disease (AD) and other rapidly progressive dementias (RPD). Serum-and CSF-tau levels were determined in patients with sporadic CJD (n = 12), AD (n = 10) and RPD but no CJD (non-CJD-RPD; n =9) who showed RPD fulfilling the WHO criteria for possible CJD at onset and had a final diagnosis other than CJD. We also analyzed serum-tau levels in healthy volunteers as a control group (n = 10). Serum-as well as CSF-tau levels were significantly elevated in CJD group compared to those in AD, non-CJD-RPD and healthy control groups. Serumtau would be a simple and useful marker to distinguish CJD from AD and non-CJD-RPD, requiring further large study to confirm this.

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Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999–2008

Abstract: Surgery or blood transfusion had little effect on the incidence of sCJD.

Cited by 27 publications

References 20 publications

Transmission Properties of Atypical Creutzfeldt-Jakob Disease: a Clue to Disease Etiology?

Transmission Properties of Atypical Creutzfeldt-Jakob Disease: a Clue to Disease Etiology?

Sensitivity to Biases of Case-Control Studies on Medical Procedures, Particularly Surgery and Blood Transfusion, and Risk of Creutzfeldt-Jakob Disease

Serum tau protein as a marker for the diagnosis of Creutzfeldt-Jakob disease

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