Creutzfeldt-Jakob disease 30 years after perforating keratoplasty

Background: Creutzfeldt-Jakob disease, currently viewed as one of the prion ic diseases, occurs in by far the majority of cases sporadically, sometimes in families, and in rare instances as a transmissible disease with every c onceivable interval of latency. History and signs: This report of a 45-year-old female concerns a spongifor m encephalopathy which appeared almost 30 years after penetrating keratopla sty. The corneal material came from a 63-year-old donor with Creutzfeldt-Ja kob disease: the keratoplasty was performed at a time when the transmissibi lity of certain diseases was still unknown. Conclusion: The risk of transmission of the disease is very low but cannot be ruled out.