PT  - JOURNAL ARTICLE
AU  - J G Heckmann
AU  - C J G Lang
AU  - F Petruch
AU  - A Druschky
AU  - C Erb
AU  - P Brown
AU  - B Neundörfer
TI  - Transmission of Creutzfeldt-Jakob disease via a corneal transplant
AID  - 10.1136/jnnp.63.3.388
DP  - 1997 Sep 01
TA  - Journal of Neurology, Neurosurgery & Psychiatry
PG  - 388--390
VI  - 63
IP  - 3
4099  - http://jnnp.bmj.com/content/63/3/388.short
4100  - http://jnnp.bmj.com/content/63/3/388.full
SO  - J Neurol Neurosurg Psychiatry1997 Sep 01; 63
AB  - 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.