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Creutzfeldt–Jakob disease after receipt of a previously unimplicated brand of dura mater graft

From Epidemiology Program Office (Dr. Hannah) and National Center for Infectious Diseases (Drs. Belay and Schonberger), Centers for Disease Control and Prevention, Atlanta, GA; Case Western Reserve University (Drs. Gambetti, Parchi, and Capellari), Cleveland, OH; Florida Department of Public Health (Dr. Krause), Tallahassee, FL; and Colorado Department of Public Health and Environment (Dr. Hoffman), Denver, CO.

Address correspondence and reprint requests to Dr. E.L. Hannah, 720 Park Boulevard, Suite 120, Boise, ID 83712; e-mail: idpro.lhannah{at}sdps.org

BACKGROUND: Iatrogenic Creutzfeldt–Jakob disease (CJD) transmission via dura mater grafts has been reported in many countries. In September 1998, a 39-year-old Colorado woman was reported as having suspected CJD after receiving a dura mater graft 6 years earlier.

METHODS: An investigation was initiated to confirm the diagnosis of CJD and assess the possible source of CJD transmission. The authors determined the presence or absence of other known CJD risk factors, checked for epidemiologic evidence of possible CJD transmission via neurosurgical instruments, and evaluated the procedures used in the collection and processing of the graft, including whether the donor may have had CJD.

RESULTS: The CJD diagnosis was confirmed in the dural graft recipient by neuropathologic and immunodiagnostic evaluation of the autopsy brain tissue. She had no history of receipt of cadaveric pituitary hormones or corneal grafts or of CJD in her family. The authors found no patients who underwent a neurosurgical procedure within 6 months before or 5 months after the patient’s surgery in 1992 who had been diagnosed with CJD. The dura mater was obtained from a 57-year-old man with a history of dysarthria, ataxia, and behavioral changes of uncertain origin. The graft was commercially prepared by use of a process that included treatment with 0.1 N sodium hydroxide and avoided commingling of dura from different donors.

CONCLUSIONS: The patient’s age, absence of evidence for other sources of CJD, the latent period, and the report of an unexplained neurologic illness in the donor of the dura mater indicate that the graft was the most likely source of CJD in this patient.

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