Transmission of Creutzfeldt-Jakob disease via a corneal transplant
- aDepartment of Neurology, University of Erlangen Nuremberg, Germany, bDepartment of Neurology, Bezirkskrankenhaus Ansbach, Germany, cDepartment of Ophthalmology, University of Tübingen, Germany, dNational Institutes of Health, Laboratory of Central Nervous System Studies, Bethesda, Maryland, USA
- Dr Josef Georg Heckmann, Department of Neurology, University of Erlangen-Nuremberg, Schwabachanlage 6, D-91054 Erlangen, Germany.
- Received 24 October 1996
- Revised 7 March 1997
- Accepted 14 March 1997
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.