Manifestation einer Creutzfeldt-Jakobschen Erkrankung 30 Jahre nach einer Hornhauttransplantation¹

Zusammenfassung

Hintergrund Die Creutzfeldt-Jakobsche Erkrankung, die nach derzeitiger Auffassung den Prionenkrankheiten zugerechnet wird, tritt weit überwiegend sporadisch, gelegentlich familiär und in seltenen Fällen als übertragbare Krankheit mit höchst unterschiedlicher Latenzzeit auf.Patient Es wird über eine spongiöse Enzephalopathie berichtet, die bei einer 45-jährigen Frau nahezu 30 Jahre nach einer perforierenden Keratoplastik aufgetreten ist. Das Hornhautmaterial stammte von einer 65-jährigen Spenderin, die an einer spongiformen Enzephalopathie litt; die Keratoplastik erfolgte zu einer Zeit, als die Übertragbarkeit bestimmter Erkrankungen noch nicht bekannt war.Schlussfolgerung Das Risiko, über eine Hornhauttransplantation an einer spongiösen Enzephalopathie zu erkranken, ist zwar gering, aber grundsätzlich nicht auszuschließen.Schlüsselwörter Creutzfeldt-Jakobsche Erkrankung - Hornhaut - Hornhauttransplantation - übertragbare Infektion

Creutzfeldt-Jakob disease 30 years after perforating keratoplasty

Background Creutzfeldt-Jakob disease, currently viewed as one of the prionic diseases, occurs in by far the majority of cases sporadically, sometimes in families, and in rare instances as a transmissible disease with every conceivable interval of latency.History and signs This report of a 45-year-old female concerns a spongiform encephalopathy which appeared almost 30 years after penetrating keratoplasty. The corneal material came from a 63-year-old donor with Creutzfeldt-Jakob disease; the keratoplasty was performed at a time when the transmissibility of certain diseases was still unknown.Conclusion The risk of transmission of the disease is very low but cannot be ruled out.Key words Creutzfeldt-Jakob's disease - cornea - cornea transplantation - transmitted infection

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