Towards an age-dependent transmission model of acquired and sporadic Creutzfeldt-Jakob disease

PLoS One. 2014 Oct 3;9(10):e109412. doi: 10.1371/journal.pone.0109412. eCollection 2014.

Abstract

Introduction: Sporadic Creutzfeldt-Jakob disease (sCJD) might be transmitted by surgery. The purpose of this study was to investigate potential susceptibility to sCJD from surgery at juvenile age and in early adulthood.

Methods: From Danish and Swedish national registries we identified 167 definite and probable sCJD cases with onset from 1987 through 2003, and 835 age-, sex- and residence-matched controls along with their surgical histories. Main, anatomically or etiologically classified surgical procedures followed by a ≥20-year lag were analyzed using logistic regression, and stratified by age at first-registered surgical discharge.

Results: The risk of having a diagnosis of CJD depended strongly on age at first surgery with odds ratio (OR) of 12.80 (95% CI 2.56-64.0) in patients <30 years, 3.04 (95% 1.26-7.33) in 30-39 years, and 1.75 (95% CI 0.89-3.45) in ≥40 years, for anatomically classified surgical procedures. Similar figures were obtained for etiologically classified surgical procedures.

Conclusions: Risk of surgical-acquired sCJD depends on age at exposure; this pattern is similar to age-specific profiles reported for CJD accidentally transmitted by human pituitary-derived growth hormone and susceptibility curves for variant CJD estimated after adjustment for dietary exposure to bovine spongiform encephalopathy. There might be an age-at-exposure-related susceptibility to acquire all CJD forms, including sCJD from routine surgery.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Age Factors
  • Case-Control Studies
  • Creutzfeldt-Jakob Syndrome / epidemiology*
  • Creutzfeldt-Jakob Syndrome / mortality
  • Creutzfeldt-Jakob Syndrome / surgery
  • Creutzfeldt-Jakob Syndrome / transmission*
  • Data Interpretation, Statistical
  • Female
  • Humans
  • Logistic Models
  • Male
  • Middle Aged
  • Models, Statistical*
  • Registries
  • Risk Factors
  • Young Adult

Supplementary concepts

  • Creutzfeldt-Jakob Disease, Sporadic

Grants and funding

Funding was obtained from the EU Research Commission, Concerted Action QLRG3-CT-2002-81223, NEUROPRION and the Spanish Centro de Investigaciones en enfermedades Neurológicas (CIEN C03-06), and Consortium for Biomedical Research in Neurodegenerative Diseases (CIBERNED) networks, and from the Karolinska and Carlos III National Health Institutes. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.