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
Brown P. An epidemiologic critique of Creutzfeldt-Jakob
disease.
Epidemiol Rev 1980;2:113-35.
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.
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.
Will RG, Matthews WB. Evidence for case-to-case transmission
of
Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry
1982;45:235-8.
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.
CDC. Fatal degenerative neurologic disease in patients who
received pituitary-derived human growth hormone. MMWR
1985;34:359-60, 365-6.
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.
Brown P, Gibbs CJ Jr, Amyx HL, et al. Chemical disinfection of
Creutzfeldt-Jakob disease virus. N Engl J Med
1982;306:1279-82.
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.
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.
American Association of Tissue Banks. Standards for tissue
banking. Arlington, Virginia: American Association of Tissue
Banks, 1984.
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.
Disclaimer
All MMWR HTML documents published before January 1993 are electronic conversions from ASCII text into HTML. This conversion may have resulted in character translation or format errors in the HTML version. Users should not rely on this HTML document, but are referred to the original MMWR paper copy for the official text, figures, and tables. An original paper copy of this issue can be obtained from the Superintendent of Documents, U.S. Government Printing Office (GPO), Washington, DC 20402-9371; telephone: (202) 512-1800. Contact GPO for current prices.
**Questions or messages regarding errors in formatting should be addressed to mmwrq@cdc.gov.
