Creutzfeldt-Jakob disease and the eye

Authors: Head, Mark W; Ironside, James W

Source: Expert Review of Ophthalmology, Volume 3, Number 4, August 2008 , pp. 481-490(10)

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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

Document Type: Research article

DOI: http://dx.doi.org/10.1586/17469899.3.4.481

Affiliations: 1: National Creutzfeldt-Jakob Disease Surveillance Unit, Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, EH4 2XU, UK., Email: m.w.head@ed.ac.uk

Publication date: 2008-08-01

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