NR ACKP
AU Cavanagh,H.D.; Hogan,R.N.
TI Creutzfeldt-Jakob disease
QU The New England Journal of Medicine 1999 May 27; 340(21): 1688-9
IA http://www.nejm.org/content/1999/0340/0021/1687b.asp
KZ N Engl J Med. 1998 Dec 31;339(27):1994-2004. PMID: 9869672
PT Brief an den Editor
VT
In the article by Johnson and Gibbs on prion diseases, there is only passing mention of "suspected" transmission of Creutzfeldt-Jakob disease in 1974 through a corneal transplant. The authors state further that "human transmission was more... convincingly demonstrated... after... surgery to excise epileptic foci." However, in the case of the corneal transplant, specimens from both the donor and the recipient were later reviewed by knowledgeable neuropathologists, and the diagnosis was reconfirmed. (1) Furthermore, inoculation of brain tissue from the donor produced clinical Creutzfeldt-Jakob disease in primates after extended incubation, and secondary transmission was pathologically confirmed. (2) Recently, two additional cases of probable and possible transmission have also been reported in the literature from Japan and Germany. (3)
The most important new case of the transmission of prion disease through corneal transplants, however, occurred recently in Great Britain. In February 1997, a 53-year-old woman died of lung cancer. For several weeks before her death, she was described by one of her daughters as "falling over," having a "staggering gait," and "acting like a senile old lady" (4); symptoms were attributed to presumed metastasis of the cancer to the central nervous system. In early March 1997, both of the woman's corneas were transplanted to two recipients; a third received sclera from the donor. In November 1997, the donor's brain was examined, revealing a spongiform encephalopathy typical of sporadic Creutzfeldt-Jakob disease, later confirmed by neuropathologists at the United Kingdom Creutzfeldt-Jakob Disease Surveillance Unit. The transplant recipients were notified, the transplanted tissues were removed, and surveillance continues. (3) These three cases arouse particular concern, since the infectivity (expressed as the median infective dose) of corneal tissue in scrapie, the prototypic prion disease of animals, was reported as 5.4 log units per milliliter, as compared with 8.9 log units per milliliter for brain and 8.4 log units per milliliter for retinal tissue. (3) Other animal studies have also shown that corneal tissue harbors the agent and can transmit disease. (5)
The possibility of a recent increase in iatrogenic cases of Creutzfeldt-Jakob disease resulting from the transplantation of prion-infected corneas has created heightened medical and public sensitivity regarding U.S. donor-screening practices for the more than 40,000 corneas transplanted annually. On the basis of recent events, additional measures for tightening inclusion criteria with respect to the medical history have been proposed for potential donors of ocular tissue. (3) Fortunately, the criteria introduced in the 1980s have been adequate, and with additional safeguards in place, they should ensure the best possibility of continued safety. Because of the large numbers of patients involved, however, we hope that a reliable and specific laboratory screening method will become available in the near future and will eliminate these issues of concern.
References
1. Brown P, Cervenakova L, Goldfarb LG, et al. Iatrogenic Creutzfeldt-Jakob disease: an example of the interplay between ancient genes and modern medicine. Neurology 1994;44:291-3.
2. Brown P, Gibbs CJ Jr, Rodgers-Johnson P, et al. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol 1994;35:513-29.
3. Hogan RN, Brown P, Heck E, Cavanagh HD. Risk of prion disease transmission from ocular donor tissue transplantation. Cornea 1999;18:2-11.
4. CJD and the eye. Focus: Royal College of Ophthalmologists. Autumn 1998:1.
5. Hogan RN, Cavanagh HD. Transplantation of corneal tissue from donors with diseases of the central nervous system. Cornea 1995;14:547-53.
ZR 5
MH Corneal Transplantation/*adverse effects; Creutzfeldt-Jakob Syndrome/*transmission; Female; Human; Middle Age
SP englisch
PO USA