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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.

Background: In most cases, craniosynostosis is a sporadic occurrence of unknown etiology. Efforts to ascertain the exact, cause of this deformation often fall to reveal its origin and pathogenetic mechanism. Some previous reports have described isolated instances of the association of neural tube defects (NTD) and craniosynostosis, but in none of them has a causal relationship been established to explain this coincidence, apart from a fortuitous sequence of congenital defects., Methods: We have reviewed the clinical records of 40 patients diagnosed with craniosynostosis who were studied at our hospital over a 22-year period, for the purpose of determining the possible etiologies of this cranial deformation., Results: We found that three children with unilateral coronal synostosis had associated NTD-an incidence of 7.5%. Two children had cephaloceles and the third harbored a lumbosacral myelomeningocele. In these three cases, the craniosynostosis was noted immediately after birth and before the development or treatment of hydrocephalus., Conclusion: We think that there must be an alternative explanation for the association of craniosynostosis and NTD other than overdrained hydrocephalus or chance association. We advance the hypothesis that in these cases the cause of the premature coronal synostosis was the lack of cerebral expansion. Since brain expansion and intracranial pulse pressure determine the growth and shape of the overlying cranial vault, the dampening effect of the malformation sac (myelomeningocele or cephalocele) on the intracranial pressure and on the pulse pressure may lead to close contact of the affected bones and to the premature closure of the coronal suture. The possibility of fetal head constraint because of diminished fetal activity due to the NTD as a contributory factor is also suggested.