Creutzfeldt-Jakob disease acquired via a dural graft: failure of therapy with quinacrine and chlorpromazine

Abstract

Background: Accidental transmission of Creutzfeldt-Jakob disease (CJD) has been reported after neurosurgical interventions, use of intracerebral electrodes, corneal transplants, and after the administration of human-derived hormones. Acquired CJD has also been documented after dural grafting with tissues of human cadaver origin. At present, quinacrine and chlorpromazine are being investigated for the treatment of sporadic CJD, with the hope of offering an effective treatment of an otherwise fatal disease. Our objective was to report a case of iatrogenic CJD occurring 18 years after the implant of a dural graft of human origin and to inform on the results of the treatment with quinacrine and chlorpromazine.

Case description: In 1984, a 46-year-old woman was given a Lyodura graft for decompression of Chiari I malformation and syringomyelia. The patient was diagnosed with CJD in 2002. In view of the scarce options for treatment of CJD and after reviewing the current literature, the patient was treated with quinacrine and chlorpromazine. She showed no clinical improvement and died 6 months after hospital admission. The iatrogenic origin of the disease in this patient is supported by the clinical features, laboratory data, and findings from the brain necropsy.

Conclusions: Patients who have received a dural graft of cadaveric origin may be at risk for developing CJD after very prolonged incubation periods. Treatment with quinacrine and chlorpromazine for acquired CJD was ineffective in our patient. A clinical trial on the use of antiprion agents is warranted.