Abstract
Prion diseases encompass inherited, sporadic and iatrogenic mammalian neurological disorders, and is characterized by the conversion of the cellular prion in an insoluble and protease-resistant isoform. A nationwide survey and recent information documented 70 patients with Creutzfeldt-Jakob disease (CJD) who had received cadaveric dura mater grafts during the period between January 1979 and September 1999 in Japan. At least 64 of these 70 patients received the same brand of dura mater grafts from the same processor.
Mean age at disease onset in the 70 patients with dural grafts was younger (53years) than that in patients with sporadic disorders (63years) (p<0.0001). Initial predominant symptoms were cerebellar ataxia and disoirientation.
