Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions

Abstract

ObjectivesEvidence of surgical transmission of sporadic Creutzfeldt–Jakob disease (sCJD) remains debatable in part due to misclassification of exposure levels. In a registry-based case–control study, the authors applied a risk-based classification of surgical interventions to determine the association between a history of surgery and sCJD.DesignCase–control study, allowing for detailed analysis according to time since exposure.SettingNational populations of Denmark and Sweden.ParticipantsFrom national registries of Denmark and Sweden, the authors included 167 definite and probable sCJD cases with onset during the period 1987–2003, 835 age-, sex- and residence-matched controls and 2224 unmatched. Surgical procedures were categorised by anatomical structure and presumed risk of transmission level. The authors used logistic regression to determine the odds ratio (OR) for sCJD by surgical interventions in specified time-windows before disease-onset.ResultsFrom comparisons with matched controls, procedures involving retina and optic nerve were associated with an increased risk at a latency of ≥1 year OR (95% CI) 5.53 (1.08 to 28.0). At latencies of 10 to 19 years, interventions on peripheral nerves 4.41 (1.17 to 16.6) and skeletal muscle 1.58 (1.01 to 2.48) were directly associated. Interventions on blood vessels 4.54 (1.01 to 20.0), peritoneum 2.38 (1.14 to 4.96) and skeletal muscle 2.04 (1.06 to 3.92), interventions conducted by vaginal approach 2.26 (1.14 to 4.47) and a pooled category of lower-risk procedures 2.81 (1.62 to 4.88) had an increased risk after ≥20 years. Similar results were found when comparing with unmatched controls.InterpretationThis observation is in concordance with animal models of prion neuroinvasion and is likely to represent a causal relation of surgery with a non-negligible proportion of sCJD cases.

Genetic variability of the gene cluster CALHM1–3 in sporadic Creutzfeldt-Jakob disease

“…14 Several lines of evidence suggest that AD and prion diseases share pathophysiologic mechanisms. [15][16][17][18][19][20][21][22][23] In this paper, we investigated the genetic variability of the gene cluster formed by CALHM1 and its paralogs in a Caucasian population of Spanish origin, and explored the potential association of these genes with sporadic Creutzfeldt-Jakob disease (sCJD).…”

Creutzfeldt-Jakob disease mortality in Canada, 1998 to 2013

“…Although iCJD is very rare, epidemics have been welldocumented, with 469 cases reported worldwide as of 2012 (7). In addition, carefully conducted epidemiological studies have yielded new evidence that a fraction of "sporadic" CJD cases may be attributable to remote iatrogenic exposure through surgery, particularly early in life (8,9). Most recently, a new human prion disease, variant CJD (vCJD), emerged in the mid-1990s in the United Kingdom via zoonotic transmission to humans of a prion disease of cattle, bovine spongiform encephalopathy (BSE) (10−12).…”

Sporadic and Infectious Human Prion Diseases

“…This observation is consistent with case-control studies of risk factors for the development of disease, which have been largely negative, with positive findings likely reflecting methodological biases rather than true biological risk factors (de Pedro Cuesta et al 2012). The exceptions are an increased frequency of sCJD in patients with a history of prior surgery (de Pedro Cuesta et al 2011), although this is not consistent in all studies (Ward et al 2008). There is no evidence of an increased risk through working, for example, in medical or paramedical occupations (Alcalde-Cabero et al 2012), or related to social contact with affected patients.…”