Creutzfeldt–Jakob disease and the eye

Abstract

Creutzfeldt–Jakob disease (CJD) belongs to the group of human prion diseases – rare neurodegenerative disorders that are caused by transmissible agents known as prions. In CJD, the levels of prion infectivity are highest in the brain, and there is evidence from experimental models that similar levels of infectivity are likely to be present in the retina and optic nerve. CJD has been transmitted by corneal transplantation and there have been increasing concerns that transmission by ophthalmic surgery (particularly retinal surgery) is a possibility. This article outlines the current information on ocular tissue involvement in CJD, discusses concerns regarding the risk of potential transmission and addresses current issues for risk management in ophthalmic surgery.

Keywords::

• corneal transplantation
• decontamination
• iatrogenic CJD
• infectivity
• optic nerve
• prion protein
• retina
• sporadic CJD
• variant CJD

Acknowledgements

We are grateful to clinicians, pathologists and laboratory and mortuary staff across the UK for referring cases of suspected CJD to the National Creutzfeldt–Jakob Disease Surveillance Unit, and would like to thank relatives of patients for giving consent to undertake tissue-based research.

Financial & competing interests disclosure

The National CJD Surveillance Unit is funded by the Department of Health and the Scottish Government. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.