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Clinical science
Scientific reports
Abnormal prion protein in the retina of the most commonly occurring subtype of sporadic Creutzfeldt-Jakob disease
  1. M W Head1,
  2. A H Peden1,
  3. H M Yull1,
  4. D L Ritchie1,
  5. R E Bonshek2,
  6. A B Tullo2,
  7. J W Ironside1
  1. 1National CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK
  2. 2Academic Department of Ophthalmology, Manchester Royal Eye Hospital, Manchester M13 9WH, UK
  1. Correspondence to: Dr M W Head National CJD Surveillance Unit, Bryan Matthews Building, Western General Hospital, Edinburgh EH4 2XU, UK; m.w.headed.ac.uk

Abstract

Background: Involvement of the eye has been reported in patients with variant Creutzfeldt-Jakob disease (vCJD), but there is disagreement on whether retinal involvement occurs in sporadic Creutzfeldt-Jakob disease (sCJD).

Methods: Western blotting, paraffin embedded tissue blotting, and immunohistochemistry were used to test whether the abnormal form of the prion protein (PrPSc) accumulates to detectable levels in the eye in a case of the most common subtype of sCJD (MM1).

Results: Low levels of PrPSc were detectable in the retina, localised to the plexiform layers of the central retina. PrPSc was not detectable in other ocular tissues.

Conclusions: The abnormal form of the prion protein is present in the retina in the most common sCJD subtype (MM1), albeit at levels lower than those found previously in vCJD and in sCJD of the VV2 subtype.

  • sCJD, sporadic Creutzfeldt-Jakob disease
  • vCJD, variant Creutzfeldt-Jakob disease
  • Creutzfeldt-Jakob disease
  • prion protein
  • PRNP codon 129 genotype
  • retina
  • sCJD, sporadic Creutzfeldt-Jakob disease
  • vCJD, variant Creutzfeldt-Jakob disease
  • Creutzfeldt-Jakob disease
  • prion protein
  • PRNP codon 129 genotype
  • retina

https://doi.org/10.1136/bjo.2004.063495

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    Footnotes

    • The National CJD Surveillance Unit is funded by the UK Department of Health and the Scottish Executive and is a member of the EU TSELAB (EC QLK-CT-2002-81523) and NeuroPrion (EC FOOD-CT-2004-506579) projects.

    • Competing interests: none declared

    • Ethical approval for the acquisition and use of autopsy material for research on transmissible spongiform encephalopathies in the National CJD Surveillance Unit brain bank is covered by LREC 2000/4/157 (Professor J W Ironside).