J Neurol Neurosurg Psychiatry 1997;63:388-390 ( September )

Short report

Transmission of Creutzfeldt-Jakob disease via a corneal transplant J G Heckmann,^a C J G Lang,^a F Petruch,^b A Druschky,^a C Erb,^c P Brown,^d B Neundörfer^a

^a Department of Neurology, University of Erlangen Nuremberg, Germany, ^b Department of Neurology, Bezirkskrankenhaus Ansbach, Germany, ^c Department of Ophthalmology, University of Tübingen, Germany, ^d National Institutes of Health, Laboratory of Central Nervous System Studies, Bethesda, Maryland, USA

Correspondence to: Dr Josef Georg Heckmann, Department of Neurology, University of Erlangen-Nuremberg, Schwabachanlage 6, D-91054 Erlangen, Germany.

Received 24 October 1996 and in revised form 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.

Keywords: Creutzfeldt-Jakob disease; corneal graft; iatrogenic transmission

---

© 1997 by Journal of Neurology, Neurosurgery, and Psychiatry
CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Brown, P., Brandel, J.-P., Preese, M., Sato, T. (2006). Iatrogenic Creutzfeldt-Jakob disease: The waning of an era. Neurology 67: 389-393 [Abstract] [Full Text]  
• DeJoia, C., Moreaux, B., O'Connell, K., Bessen, R. A. (2006). Prion Infection of Oral and Nasal Mucosa. J. Virol. 80: 4546-4556 [Abstract] [Full Text]  
• Gupta, M, Singh, A D, Rundle, P A, Rennie, I G (2004). Bovine pericardium (Ocuguard) wrap for hydroxyapatite implants. Br. J. Ophthalmol. 88: 834-835 [Full Text]  
• S-Juan, P, Ward, H J T, De Silva, R, Knight, R S G, Will, R G (2004). Ophthalmic surgery and Creutzfeldt-Jakob disease. Br. J. Ophthalmol. 88: 446-449 [Full Text]  
• Will, R. G (2003). Acquired prion disease: iatrogenic CJD, variant CJD, kuru. Br Med Bull 66: 255-265 [Abstract] [Full Text]  
• Hogan, R. N., Cavanagh, H. D., Brown, P. (2003). Qualifications to the Report of a New Case of Creutzfeldt-Jakob Disease in the Recipient of a Corneal Transplant. Arch Neurol 60: 293-294 [Full Text]  
• Head, M. W., Northcott, V., Rennison, K., Ritchie, D., McCardle, L., Bunn, T. J. R., McLennan, N. F., Ironside, J. W., Tullo, A. B., Bonshek, R. E. (2003). Prion Protein Accumulation in Eyes of Patients with Sporadic and Variant Creutzfeldt-Jakob Disease. IOVS 44: 342-346 [Abstract] [Full Text]  
• Mehta, J S, Franks, W A (2002). The sclera, the prion, and the ophthalmologist. Br. J. Ophthalmol. 86: 587-592 [Abstract] [Full Text]  
• Gupta, M, Puri, P, Rennie, I G (2002). Use of bovine pericardium as a wrapping material for hydroxyapatite orbital implants. Br. J. Ophthalmol. 86: 288-289 [Abstract] [Full Text]  
• Bednarz, J., Doubilei, V., Wollnik, P. C M, Engelmann, K. (2001). Effect of three different media on serum free culture of donor corneas and isolated human corneal endothelial cells. Br. J. Ophthalmol. 85: 1416-1420 [Abstract] [Full Text]  
• Kennedy, R. H., Hogan, R. N., Brown, P., Holland, E., Johnson, R. T., Stark, W., Sugar, J. (2001). Eye Banking and Screening for Creutzfeldt-Jakob Disease. Arch Ophthalmol 119: 721-726 [Abstract] [Full Text]  
• Brown, P., Preece, M., Brandel, J.-P., Sato, T., McShane, L., Zerr, I., Fletcher, A., Will, R. G., Pocchiari, M., Cashman, N. R., d'Aignaux, J. H., Cervenakova, L., Fradkin, J., Schonberger, L. B., Collins, S. J. (2000). Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology 55: 1075-1081 [Abstract] [Full Text]  
• Kropp, S., Schulz-Schaeffer, W. J., Finkenstaedt, M., Riedemann, C., Windl, O., Steinhoff, B. J., Zerr, I., Kretzschmar, H. A., Poser, S. (1999). The Heidenhain Variant of Creutzfeldt-Jakob Disease. Arch Neurol 56: 55-61 [Abstract] [Full Text]  
• Allan, B., Tuft, S. (1997). Transmission of Creutzfeldt-Jakob disease in corneal grafts. BMJ 315: 1553-1554 [Full Text]