Abnormal prion protein in the retina of the most commonly occurring subtype of sporadic Creutzfeldt-Jakob disease

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.