PrPres deposition in the retina is a common finding of sporadic, familial and iatrogenic Creutzfeldt-Jakob diseases (CJD)

Head MW, Northcott V, Rennison K, Ritchie D, McCardle L, Bunn TJR, McLennan NF, Ironside JW, Tullo AB, Bonshek RE (2003) Prion protein accumulation in eyes of patients with sporadic and variant Creutzfeldt-Jakob disease. Invest Opthalmol Vis Sci:44. https://doi.org/10.1167/iovs.01-1273 Head MW, Peden AH, Yull HM, Ritchie DL, Bonshek RE, Tullo AB, Ironside JW (2005) Abnormal prion protein in the retina of the most commonly occurring subtype of sporadic Creutzfeldt-Jakob disease. Br J Ophthalmol 89:1131–1133. https://doi.org/10.1136/bjo.2004.063495 Takao M, Kimura H, Mihara B (2017) How can we increase the number of autopsies for prion diseases? A model system in Japan. J Neurol Sci 373:58–59. https://doi.org/10.1016/j.jns.2016.11.073 Parchi P, Giese A, Capellari S, Brown P, Schulz-Schaeffer W, Windl O, Zerr I, Budka H, Kopp N, Piccardo P, Poser S, Rojiani A, Streichemberger N, Julien J, Vital C, Ghetti B, Gambetti P, Kretzschmar H (1999) Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 46:224–233 Parchi P, Strammiello R, Notari S, Giese A, Langeveld JP, Ladogana A, Zerr I, Roncaroli F, Cras P, Ghetti B, Pocchiari M, Kretzschmar H, Capellari S (2009) Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification. Acta Neuropathol 118:659–671. https://doi.org/10.1007/s00401-009-0585-1 Wadsworth JD, Joiner S, Hill AF, Campbell TA, Desbruslais M, Luthert PJ, Collinge J (2001) Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay. Lancet 358:171–180 Head MW, Ironside JW, Ghetti B, Jeffrey M, Piccardo P, Will R, G. (2015) Prion diseases. In: Love S, Perry A, Ironside JW, Budka H (eds) Greenfield’s neuropathology 9th edn. CRC Press, City, pp 1016–1086 Koronyo Y, Biggs D, Barron E, Boyer DS, Pearlman JA, Au WJ, Kile SJ, Blanco A, Fuchs D-T, Ashfaq A, Frautschy S, Cole GM, Miller CA, Hinton DR, Verdooner SR, Black KL, Koronyo-Hamaoui M (2017) Retinal amyloid pathology and proof-of-concept imaging trial in Alzheimer’s disease. JCI Insight 2:e93621. https://doi.org/10.1172/jci.insight.93621