OBJECTIVES:

Creutzfeldt-Jakob disease (CJD) is a progressive, fatal disease featuring severe neurological symptoms. Most people with CJD will die within a year of initial presentation. Whilst rare, incidences of iatrogenic CJD (iCJD) have been recorded for medical procedures including neurosurgery as CJD prions are unlikely to be completely by conventional hospital sterilisation techniques and can remain on surgical instruments. Additionally, CJD reaches high levels of infectivity in the brain and can incubate silently in humans for up to 40 years. In 2005 the UK National Institute for Health and Care Excellence (NICE) issued guidance, informed by ScHARR, to tighten decontamination procedures for neurosurgical instruments. NICE have re-commissioned ScHARR to systematically review the evidence, incorporating new information since 2005.

METHODS:

Eight systematic reviews were conducted. Four questions were fundamental to understanding CJD disease including: (1) prevalence and incidence, (2) risk of transmission via surgery, (3) incubation periods and (4) infectivity. Four further reviews were undertaken to understand the risks of transmission including: (5) efficacy of current decontamination procedures, (6) adherence to NICE guidance to keep surgical instrument sets together, (7) evidence of complications from single-use instruments and (8) likelihood of patients returning for further surgery.

RESULTS:

Published direct evidence identified from 8776 records indicates that the risks of iCJD via surgery are low but that the general rate of CJD cases is increasing worldwide. Multiple reasons are posited. A paucity of direct evidence in humans to inform the research question on the risks and benefits of re-usable versus single-use instruments was noted. The data from relevant studies are presented. Data requirements and the implications for policy-making are discussed.

CONCLUSIONS:

Comprehensive, targeted methods to answer this multi-faceted question are crucial to health care use and policy relating to CJD worldwide. Our findings highlight the challenges of informing evidence-based decisions in rare but highly infectious diseases.