Creutzfeldt-Jakob Disease Transmitted by Dura mater Graft

We report a 41-year-old woman who underwent a craniotomy and removal of a left clinoidal meningioma invading the cavernous sinus in May 1989. A graft of cadaveric dura mater (lyodura) was used as plastia, covering part of the left temporal lobe. A small tumoral fragment remaining was treated with radiotherapy. She stayed asymptomatic until October 1992 (41 months after surgery) when she began a rapidly progressive clinical deterioration (ataxia, dysarthria, pyramidalism, myoclonus and generalized slowing on the EEG). Two months later, a brain tissue frontal biopsy (fig. 1) led to the diagnosis of spongiform encephalopathy, thus suggesting Creutzfeldt-Jakob disease (CJD). The patient went into a progressive coma dying in February 1993. The first description of a transmissible, person-to-person form of CJD was reported in 1974 in a patient who had received a corneal transplant [1]. Thadani et al. [2] published the first report of CJD transmitted by a cadaveric dura mater graft in 1988. Since then, 14 more cases [3–5] including ours have been reported (table 1). All of them, except 1, related to a commercial dura mater (Lyodura) made by Brown Melsungen Company of Germany. CJD can be transmitted through particles containing an abnormal form of a prion protein. These infectious particles are highly resistant to most of the common sterilization procedures. The Committee on Health Care Issues of the American Neurological Association [6] recommended 1-hour exposure to 1 molar NaOH, or steam autoclaving for 1 h at 132 ° C as the standard sterilization procedures for CJD tissue or contaminated material. After May 1987, according