CREUTZFELDT-JAKOB-DISEASE IN AUSTRIA

Between 1969 and 30th June 1996, Creutzfeldt-Jakob disease (CJD) was d efinitively diagnosed in 88 Austrian patients by autopsy and/or biopsy . The number of diagnosed cases has steadily increased in recent years (average incidence in 1969-1985: 0.18 per million; 1986-1994: 0.67 pe r million; 1995: 1.25 per million; estimate for 1996: 1.7 per million) . The percentage of patients older than 70 years increased until 1989 and has decreased slightly since then. One patient received a lyophili sed dura transplant 11 years before death. Another patient gave a hist ory of intramuscular injections of bovine RNA (Regeneresen(R)) extract ed from various organs including brain administered over a ten-year pe riod. One female patient had familial CJD with a glutamate --> lysine mutation at codon 200 of the prion protein (PrP) gene PRNP. The ages a t death are distributed symmetrically around the median of 64 years. T wo female patients died at the unusually young ages of 27 and 30 years . Median duration of disease was 4.5 months; 77% of the patients died within 6 months after onset of the disease. Retrospectively, 83% of th e patients fulfilled the clinical criteria of probable or possible CJD . Neuropathologically, each patient showed accumulation of immunocytoc hemically detectable pathological PrP in the central nervous system. N o patient had the neuropathological profile of the new CJD variant rec ently described in the U.K. The recent rise in diagnosed CJD in Austri a probably reflects increased awareness and recognition rather than a real increase. Since bovine spongiform encephalopathy (BSE) has not be en observed in Austria, our data argue against an association between the rise of incidence of CJD and BSE.