Transmission of Creutzfeldt-Jakob disease via a corneal transplant

TitleTransmission of Creutzfeldt-Jakob disease via a corneal transplant
Publication TypeJournal Article
Year of Publication1997
AuthorsHeckmann JG, Lang CJ, Petruch F, Druschky A, Erb C, Brown P, Neundorfer B
JournalJ Neurol Neurosurg Psychiatry
Pagination388 - 90
Date PublishedSep
ISSN0022-3050 (Print) 0022-3050 (Linking)
Accession Number9328261
KeywordsCorneal Transplantation / *adverse effects, Creutzfeldt-Jakob Syndrome / *transmission, Fatal Outcome, Female, Humans, Middle Aged

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.

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