[Infectious prion disease: CJD with dura mater transplantation]

Abstract

Prion diseases include scrapie, BSE and CWD in animals, and spontaneous, familiar and infectious Creutzfeldt-Jakob disease(CJD) in human. Infectious prion diseases include kuru, variant CJD and iatrogenic CJD. CJD has been transmitted from human to human by contaminated cadaveric dura mater grafts and by cadaveric pituitary hormones. To date, CJD associated with dura mater grafts, reaching 156 cases, has been reported in 17 countries. More 2/3 of cases have been reported in Japan. Nationwide survey and recent information documented 105 dura-related cases during the period between 1979 and 2003. At least 91 cases received same brand of dura mater by a single German company. Age at disease onset in cases with dura-related CJD was younger (54.2 +/- 14.2 years) than sporadic CJD (64 +/- 10 years). Two groups of dura-related CJD are manifest in clinical course and pathological characteristics, such as rapidly progressive group and slowly progressive. Rapidly group was similar to cases with classical CJD in clinical features and shot duration to death from onset. Slowly progressive group developed akinetic mutism longer than 1 year, and characterized by florid plaques in the brain (Kitamoto).