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Qualifications to the Report of a New Case of Creutzfeldt-Jakob Disease in the Recipient of a Corneal Transplant

Qualifications to the Report of a New Case of Creutzfeldt-Jakob Disease in the Recipient of a... Rabinstein et al1 report unusual magnetic resonance imaging findings in a case of Creutzfeldt-Jakob Disease (CJD). They suggest that their case was iatrogenic because the patient had received 3 prior corneal transplants. Although they state, "Confirmation of horizontal transmission of CJD is not possible . . . since we were unable to identify the corneal donors," they present their magnetic resonance imaging findings without qualification as the first reported "in iatrogenic CJD."1(p639) The issue of iatrogenic transmission of CJD by corneal transplantation is not trivial. Variant CJD in the United Kingdom has stimulated concern for the safety of the US corneal donor supply from both federal and professional organizations. Any bona fide transmission of CJD by this route would raise an alarm with numerous repercussions. In 1974, one verified transmission of CJD by corneal transplantation was reported.2 Subsequently, strict medical history criteria were established rejecting any donor with a personal or family history of CJD, undetermined neurologic disease, or dementia.3 Despite 600 000 procedures, no further corneal transmission has been reported in the United States for the last 28 years. A probable case of transmission was reported in Germany in a patient who 30 years earlier had received a cornea from a patient with sporadic CJD.4 A third possible case was reported in Japan in a patient with CJD who had undergone corneal transplant surgery 55 months prior to death. The authors postulated that CJD was acquired through this surgery, although no data on the medical history of the donor were provided.5 The inadequacy of proof in this case has been discussed at length.3,6,7 The article by Rabinstein et al1 falls into this same category of unproven association. That their patient had 3 corneal transplant procedures is not unusual and does not significantly increase the risk of CJD transmission.3,6 Between 35 000 and 40 000 corneal transplants occur annually in the United States. Last year, 3830 patients (12.1%) received repeated corneal transplants.8 At worst and assuming no historical screening or biological transmission restrictions,3 the risk of CJD in a US corneal donor is approximately 0.01%.3,6 Undergoing 3 procedures increases this risk to approximately 0.03%. Historical exclusion further reduces the risk. Despite multiple surgical procedures, the probability of CJD transmission from a US corneal donor is negligible. Tissue donors are closely tracked. Unless the donors in the authors' article were from cases reported by medical examiners, considerable information should be available regarding their health histories. Furthermore, most medical examiner cases involve persons 40 years or younger, and the likelihood that such donors would have CJD is low (95% of CJD cases occur in patients older than 50 years).6 The authors must provide more information on the donors' ages, causes of death, and medical histories if their assertions of iatrogenic causation are to be credible. Finally, the clinical characteristics of the case, although highly suggestive, are not sufficient to establish a diagnosis of CJD. The initial symptoms were very unusual, the electroencephalogram did not show typical 1- to 2-Hz periodic sharp waves, and the cerebrospinal fluid was not tested for the presence of 14-3-3 protein. In these circumstances, documentation of microscopic neuropathological examination and prion protein immunostaining are essential to confirm the diagnosis. Because of these considerations, we feel that this article does not provide enough evidence to claim iatrogenic transmission of CJD by corneal transplantation. Although the magnetic resonance imaging findings are interesting, their relationship to iatrogenic disease remains undetermined. References 1. Rabinstein AAWhiteman MLShebert RT Abnormal diffusion-weighted magnetic resonance imaging in Creutzfeldt-Jakob disease following corneal transplantations. Arch Neurol.2002;59:637-639.Google Scholar 2. Duffy PWolf JCollins GDeVoe AGStreen BCowen D Possible person-to-person transmission of Creutzfeldt-Jakob disease. N Engl J Med.1974;290:692-693.Google Scholar 3. Hogan RNBrown PHeck ECavanagh HD Risk of prion disease transmission from ocular donor tissue transplantation. Cornea.1999;18:2-11.Google Scholar 4. Heckmann JGLang CJPetruch F et al Transmission of Creutzfeldt-Jakob disease via a corneal implant. J Neurol Neurosurg Psychiatry.1997;63:388-390.Google Scholar 5. Uchiyama KIshido CYago SKuramaya HKimoto T An autopsy case of Creutzfeldt-Jakob disease associated with corneal transplantation [in Japanese]. Dementia (Jpn).1994;8:466-473.Google Scholar 6. Kennedy RHHogan RNBrown P et al Eye banking and screening for Creutzfeldt-Jakob Disease. Arch Ophthalmol.2001;119:721-726.Google Scholar 7. FDA Advisory Committee on Transmissible Spongiform Encephalopathies CJD transmission by corneal transplantation [transcript]. January 18, 2001. Available at: http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t1_03.pdf. Last accessed November 8, 2002. 8. Aiken-O'Neill PMannis MJ Summary of corneal transplant activity. Cornea.2002;21:1-3.Google Scholar http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Qualifications to the Report of a New Case of Creutzfeldt-Jakob Disease in the Recipient of a Corneal Transplant

Qualifications to the Report of a New Case of Creutzfeldt-Jakob Disease in the Recipient of a Corneal Transplant

Abstract

Rabinstein et al1 report unusual magnetic resonance imaging findings in a case of Creutzfeldt-Jakob Disease (CJD). They suggest that their case was iatrogenic because the patient had received 3 prior corneal transplants. Although they state, "Confirmation of horizontal transmission of CJD is not possible . . . since we were unable to identify the corneal donors," they present their magnetic resonance imaging findings without qualification as the first reported "in iatrogenic...
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Publisher
American Medical Association
Copyright
Copyright © 2003 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.60.2.293-a
Publisher site
See Article on Publisher Site

Abstract

Rabinstein et al1 report unusual magnetic resonance imaging findings in a case of Creutzfeldt-Jakob Disease (CJD). They suggest that their case was iatrogenic because the patient had received 3 prior corneal transplants. Although they state, "Confirmation of horizontal transmission of CJD is not possible . . . since we were unable to identify the corneal donors," they present their magnetic resonance imaging findings without qualification as the first reported "in iatrogenic CJD."1(p639) The issue of iatrogenic transmission of CJD by corneal transplantation is not trivial. Variant CJD in the United Kingdom has stimulated concern for the safety of the US corneal donor supply from both federal and professional organizations. Any bona fide transmission of CJD by this route would raise an alarm with numerous repercussions. In 1974, one verified transmission of CJD by corneal transplantation was reported.2 Subsequently, strict medical history criteria were established rejecting any donor with a personal or family history of CJD, undetermined neurologic disease, or dementia.3 Despite 600 000 procedures, no further corneal transmission has been reported in the United States for the last 28 years. A probable case of transmission was reported in Germany in a patient who 30 years earlier had received a cornea from a patient with sporadic CJD.4 A third possible case was reported in Japan in a patient with CJD who had undergone corneal transplant surgery 55 months prior to death. The authors postulated that CJD was acquired through this surgery, although no data on the medical history of the donor were provided.5 The inadequacy of proof in this case has been discussed at length.3,6,7 The article by Rabinstein et al1 falls into this same category of unproven association. That their patient had 3 corneal transplant procedures is not unusual and does not significantly increase the risk of CJD transmission.3,6 Between 35 000 and 40 000 corneal transplants occur annually in the United States. Last year, 3830 patients (12.1%) received repeated corneal transplants.8 At worst and assuming no historical screening or biological transmission restrictions,3 the risk of CJD in a US corneal donor is approximately 0.01%.3,6 Undergoing 3 procedures increases this risk to approximately 0.03%. Historical exclusion further reduces the risk. Despite multiple surgical procedures, the probability of CJD transmission from a US corneal donor is negligible. Tissue donors are closely tracked. Unless the donors in the authors' article were from cases reported by medical examiners, considerable information should be available regarding their health histories. Furthermore, most medical examiner cases involve persons 40 years or younger, and the likelihood that such donors would have CJD is low (95% of CJD cases occur in patients older than 50 years).6 The authors must provide more information on the donors' ages, causes of death, and medical histories if their assertions of iatrogenic causation are to be credible. Finally, the clinical characteristics of the case, although highly suggestive, are not sufficient to establish a diagnosis of CJD. The initial symptoms were very unusual, the electroencephalogram did not show typical 1- to 2-Hz periodic sharp waves, and the cerebrospinal fluid was not tested for the presence of 14-3-3 protein. In these circumstances, documentation of microscopic neuropathological examination and prion protein immunostaining are essential to confirm the diagnosis. Because of these considerations, we feel that this article does not provide enough evidence to claim iatrogenic transmission of CJD by corneal transplantation. Although the magnetic resonance imaging findings are interesting, their relationship to iatrogenic disease remains undetermined. References 1. Rabinstein AAWhiteman MLShebert RT Abnormal diffusion-weighted magnetic resonance imaging in Creutzfeldt-Jakob disease following corneal transplantations. Arch Neurol.2002;59:637-639.Google Scholar 2. Duffy PWolf JCollins GDeVoe AGStreen BCowen D Possible person-to-person transmission of Creutzfeldt-Jakob disease. N Engl J Med.1974;290:692-693.Google Scholar 3. Hogan RNBrown PHeck ECavanagh HD Risk of prion disease transmission from ocular donor tissue transplantation. Cornea.1999;18:2-11.Google Scholar 4. Heckmann JGLang CJPetruch F et al Transmission of Creutzfeldt-Jakob disease via a corneal implant. J Neurol Neurosurg Psychiatry.1997;63:388-390.Google Scholar 5. Uchiyama KIshido CYago SKuramaya HKimoto T An autopsy case of Creutzfeldt-Jakob disease associated with corneal transplantation [in Japanese]. Dementia (Jpn).1994;8:466-473.Google Scholar 6. Kennedy RHHogan RNBrown P et al Eye banking and screening for Creutzfeldt-Jakob Disease. Arch Ophthalmol.2001;119:721-726.Google Scholar 7. FDA Advisory Committee on Transmissible Spongiform Encephalopathies CJD transmission by corneal transplantation [transcript]. January 18, 2001. Available at: http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t1_03.pdf. Last accessed November 8, 2002. 8. Aiken-O'Neill PMannis MJ Summary of corneal transplant activity. Cornea.2002;21:1-3.Google Scholar

Journal

Archives of NeurologyAmerican Medical Association

Published: Feb 1, 2003

Keywords: creutzfeldt-jakob disease,keratoplasty

References