Journal Article

Transmission of Creutzfeldt-Jakob disease via a corneal transplant

Petruch, F, Druschky, A, Neundörfer, B, Heckmann, J G, Lang, C J G, Erb, C, Brown, P

1997

Overview

A 45 year old woman is reported who initially presented with a cerebellar syndrome, severe ataxia, and dysarthria. She rapidly deteriorated to coma vigile with bilateral myoclonic jerks, flexion rigidity, and immobility necessitating complete nursing. Her EEG showed generalised slow activity and periodic biphasic and triphasic waves. The CSF concentration of neuron specific enolase was very high. Consequently the diagnosis of Creutzfeldt-Jakob disease was established. Eight months later she died of respiratory complications. Thirty years earlier the patient had undergone corneal transplantation for keratoconus. Review of the organ donor’s hospital records showed that death was caused by intercurrent pneumonia subsequent to subacute spongiform encephalopathy confirmed by necropsy. In view of two previous case reports in the literature it is presumed that the cadaveric cornea was the source of transmission of Creutzfeldt-Jakob disease in this patient.

• Publisher: BMJ Publishing Group Ltd,BMJ,BMJ Publishing Group LTD,BMJ Group
• Subject: Ataxia / Biological and medical sciences / Blood & organ donations / corneal graft / Corneal transplantation / Corneal Transplantation - adverse effects / Creutzfeldt-Jakob disease / Creutzfeldt-Jakob Syndrome - transmission / Dysarthria / Fatal Outcome / Female / Hospitals / Humans / Iatrogenesis / iatrogenic transmission / Medical sciences / Metabolism / Middle Aged / Short Report / Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases / Surgery of the eye and orbit / Vitamin deficiency