PEDIATRIC CREUTZFELDT-JAKOB-DISEASE - PROBABLE TRANSMISSION BY A DURAL GRAFT

A 10-year-old boy underwent a posterior fossa craniectomy for removal of a grade 2 cerebellar astrocytoma. Dural closure was achieved by the placement of a dural graft. Eight years later the patient developed d ementia and myoclonus. Electroencephalography demonstrated generalized slow activity that evolved into a pattern of periodic triphasic waves . Computed tomography scan and magnetic resonance imaging were unremar kable. Brain biopsy confirmed spongiform encephalopathy of the Creutzf eldt-Jakob type. In the light of previous reports of four similar occu rrences, and of our own experience with two further cases of this dise ase, we believe that the cadaveric dura was the source of transmission of Creutzfeld-Jakob disease in our patient. The authors remark the im portance of the awareness of this late complication of dural substitut es, both for the diagnosis of possible future cases and for taking pre ventive measures to stop the spread of the disease.