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Epidemiologic Notes and Reports Rapidly Progressive Dementia in a Patient Who Received a Cadaveric Dura Mater Graft

In mid-November 1986, a 28-year-old woman developed gait ataxia 19 months after surgical resection of a cholesteatoma*. During surgery she received an imported, commercially prepared, human dura mater graft (LYODURA, Lot #2105, processed in 1982 by B. Braun Melsungen AG of the Federal Republic of Germany). By early December, she required assistance with ambulation and had developed dysarthria. Two weeks later she gave inappropriate responses to questions and developed visual hallucinations. By early January 1987, she developed myoclonic jerks and, on physical examination, was demented. Diagnosis of Creutzfeldt-Jakob disease (CJD) was confirmed by brain biopsy which demonstrated spongiform encephalopathy. She had no family history of degenerative neurologic disease, nor had she received cadaveric, pituitary-derived human growth hormone (HGH). No patient with known CJD had surgery in the same neurosurgical suite in the 3 months prior to this woman's operation. Reported by J Prichard, MD, V Thadani, MD, R Kalb, MD, E Manuelidis, MD, Yale University School of Medicine, New Haven, Connecticut, J Hadler, MD, MPH, State Epidemiologist, Connecticut State Dept of Health Svcs; Food and Drug Administration; Hospital Infections Program, Div of Viral Diseases, Center for Infectious Diseases, CDC.

Editorial Note

Editorial Note: CJD occurs with a frequency of about 1/1,000,000 population per year in the United States and in various populations worldwide (1). Most cases occur spontaneously in patients 50 years of age; CJD is rare in persons 30 years of age (1). Iatrogenic transmission of CJD has occurred in one patient by corneal transplant from an infected donor (2), in two patients who were exposed to intracerebral electrodes after they had been used in a CJD patient (3), in four patients in neurosurgical suites following procedures on CJD patients (4,5), and in four recipients of HGH (6,7). Onsets of symptoms following direct brain or eye exposure to the CJD agent have ranged from 16 to 28 months; however, patients who received systemic HGH have had onsets of symptoms after 4 to 21 years. No other reports of CJD transmission via dura mater grafts have been identified.

Dura mater harvested from cadavers is used predominantly in neurosurgical procedures, but is also used in orthopedic, otologic, dental, urologic, gynecologic, and cardiac procedures. Although the number of recipients of LYODURA and other dura mater grafts is not well known, the age of this patient and the 19-month interval between her graft and onset of symptoms of CJD strongly suggest that the dural graft was the vehicle for transmission of the CJD agent. The Food and Drug Administration and the Centers for Disease Control are continuing to investigate the association.

Procedures used to sterilize cadaveric dura such as exposure to ethylene oxide or ionizing irradiation are not sufficient to completely inactivate the CJD agent (8,9,10). Until methods to eliminate the CJD agent from dura mater can be better defined, the transmission of this lethal, degenerative neurological disease remains a possibility. Surgeons may wish to consider the alternative use of autologous fascia lata or temporalis fascia or of synthetic substitutes. Physicians who use cadaveric dura mater should verify that their sources follow stringent donor selection procedures and criteria such as those promulgated by the American Association of Tissue Banks (11,12).

Previous and current patients who have rapidly progressive dementing illnesses consistent with CJD and who have received a dural graft during an operative procedure should be reported, through their state health departments, to R. Janssen, MD, Division of Viral Diseases, Building 6, Room 127, Centers for Disease Control, 1600 Clifton Road, Atlanta, Georgia; telephone number (404)329-3091. Any facility finding remaining stock of LYODURA, Lot # 2105, should contact Dr. Janssen immediately regarding its disposition and possible testing.

References

  1. Brown P. An epidemiologic critique of Creutzfeldt-Jakob disease. Epidemiol Rev 1980;2:113-35.

  2. Duffy P, Wolf J, Collins G, DeVoe AG, Streeten B, Cowen D. Possible person-to-person transmission of Creutzfeldt-Jakob disease (Letter). N Engl J Med 1974;290:692-3.

  3. Bernoulli C, Siegfried J, Baumgartner G, et al. Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery (Letter). Lancet 1977;i:478-9.

  4. Will RG, Matthews WB. Evidence for case-to-case transmission of Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 1982;45:235-8.

  5. Foncin J, Gaches J, Cathala F, et al. Transmission iatrogene interhumaine possible de maladie de Creutzfeldt-Jakob avec atteinte des grains du cervelet (Abstract). Rev Neurol (Paris) 1980; 136:280.

  6. CDC. Fatal degenerative neurologic disease in patients who received pituitary-derived human growth hormone. MMWR 1985;34:359-60, 365-6.

  7. Powell-Jackson J, Weller RO, Kennedy P, Preece MA, Whitcombe EM, Newsom-Davis J. Creutzfeldt-Jakob disease after administration of human growth hormone. Lancet 1985;ii:244-6.

  8. Brown P, Gibbs CJ Jr, Amyx HL, et al. Chemical disinfection of Creutzfeldt-Jakob disease virus. N Engl J Med 1982;306:1279-82.

  9. Committee on Health Care Issues, American Neurological Association. Precautions in handling tissues, fluids, and other contaminated materials from patients with documented or suspected Creutzfeldt-Jakob disease. Ann Neurol 1986;19:75-7.

  10. Asher DM, Gibbs CJ Jr, Gajdusek DC. Slow viral infections: safe handling of the agents of subacute spongiform encephalopathies. In: Laboratory safety: principles and practices. BM Miller, ed. Washington, DC: American Society for Microbiology, 1986:59-71.

  11. American Association of Tissue Banks. Standards for tissue banking. Arlington, Virginia: American Association of Tissue Banks, 1984.

  12. American Association of Tissue Banks. Technical manual: Musculoskeletal Council. Arlington, Virginia: American Association of Tissue Banks, (in press). *A cholesteatoma is a cyst-like mass with a lining of stratified squamous epithelium; it occurs most commonly in the middle ear or mastoid region.



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