Risk factors for sporadic Creutzfeldt–Jakob disease

Authors

  • Hester J. T. Ward FFPH,

    Corresponding author
    1. National Creutzfeldt–Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom
    • National CJD Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, United Kingdom
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  • Dawn Everington MSc,

    1. National Creutzfeldt–Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom
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  • Simon N. Cousens MA,

    1. Department of Epidemiology and Population Health, London School of Hygiene and Tropical Medicine, London, United Kingdom
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  • Blaire Smith-Bathgate RGN,

    1. National Creutzfeldt–Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom
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  • Michelle Gillies MRCP,

    1. National Creutzfeldt–Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom
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  • Katy Murray MRCP,

    1. National Creutzfeldt–Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom
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  • Richard S. G. Knight FRCPE,

    1. National Creutzfeldt–Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom
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  • Peter G. Smith DSc,

    1. Department of Epidemiology and Population Health, London School of Hygiene and Tropical Medicine, London, United Kingdom
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  • Robert G. Will FRCP

    1. National Creutzfeldt–Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom
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Abstract

Objective

Although surgical transmission of Creutzfeldt–Jakob disease (CJD) has been demonstrated, these iatrogenic cases account for only a small proportion of all CJD cases. The majority are sporadic CJD (sCJD) cases of unknown cause. This study investigated whether some cases classified as sCJD might have an unrecognized iatrogenic basis through surgical or other medical procedures

Methods

This study compared medical risk factors from 431 sCJD cases referred 1998 to 2006 with 454 population control subjects. Possible geographic and temporal links between neurological and gynecological operations in 857 sCJD cases referred from 1990 to 2006 were investigated

Results

A reported history of ever having undergone surgery was associated with increased risk for sCJD (odds ratio, 2.0; 95% confidence interval, 1.3–2.1; p = 0.003). Increased risk was not associated with surgical categories chosen a priori but was confined to the residual category “other surgery,” in which the increase in risk appeared most marked for three subcategories: skin stitches, nose/throat operations, and removal of growths/cysts/moles. No convincing evidence was found of links (same hospital, within 2 years) between cases undergoing neurosurgery or gynecological surgery

Interpretation

It is unlikely that a high proportion of UK sCJD cases are the result of transmission during surgery, but we cannot exclude the possibility that such transmission occurs occasionally. A study based on accurate surgical histories obtained from medical records is required to determine whether the increased risk associated with reported surgical history reflects a causal association or recall bias. Ann Neurol 2007

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