Creutzfeldt-Jakob disease in recipients of corneal transplants.
OBJECTIVE
Creutzfeldt-Jakob disease (CJD) transmission has been documented to occur from the use of corneal grafts. We report 4 cases of CJD with a history of corneal transplantation and assess the frequency of coincidental CJD among corneal transplant recipients.


METHODS
Medical records and eye bank documents were reviewed. Genetic and neuropathologic tests on available specimens were performed at the National Prion Disease Pathology Surveillance Center. Statistical analyses were used to determine the expected number of coincidental CJD cases among the US population with a history of corneal transplantation.


RESULTS
Four CJD decedents with histories of corneal transplantation were identified: 3 from the United States and 1 from Japan. The time from transplant to onset of CJD symptoms ranged from 2 years, 11 months to 18 years. Available eye bank records did not suggest evidence of neurologic illness in the donors. Using corneal transplantation and CJD death data from 1990 through 2006, statistical analyses suggest that a case of coincidental sporadic CJD will occur among the population of corneal transplant recipients approximately every 1.5 years.


CONCLUSIONS
It is likely that these 4 recipients of transplanted corneas had sporadic CJD. Because of the many corneal transplantations performed each year in the United States, occasional cases of sporadic CJD in this population are expected.
Maddox
Ryan A
RA
Division of Viral and Rickettsial Diseases, National Center for Zoonotic, Vector-Borne, and Enteric Diseases, Centers for Disease Control and Prevention, Atlanta, GA 30333, USA. Rmaddox@cdc.gov
Belay
Ermias D
ED
Curns
Aaron T
AT
Zou
Wen-Quan
WQ
Nowicki
Scott
S
Lembach
Richard G
RG
Geschwind
Michael D
MD
Haman
Aissa
A
Shinozaki
Naoshi
N
Nakamura
Yosikazu
Y
Borer
Mark J
MJ
Schonberger
Lawrence B
LB
D000368:Aged; D016039:Corneal Transplantation / Q000009:adverse effects*; D007562:Creutzfeldt-Jakob Syndrome / Q000635:transmission*; D018562:Disease Transmission, Infectious; D005125:Eye Banks / Q000706:statistics & numerical data; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D014019:Tissue Donors