CREUTZFELDT-JAKOB-DISEASE IN AUSTRIA

Between 1969 and 30 September 1995, 79 Austrian patients had Creutzfel dt-Jakob disease (CJD) diagnosed neuropathologically by necropsy or bi opsy. The annual incidence has significantly increased in recent years (average 0.18 per million in 1969-85, and 0.67 per million in 1986-94 ; estimate for 1995: 1.5 per million). Also, the percentage of patient s with CJD over 70 years at death increased significantly until 1989 b ut is since in decline. There is no regional clustering, familial occu rrence, or recognised iatrogenic risk. One patient had a 10 year histo ry of intramuscular injection of purified bovine RNA preparation (Rege neresen(R)) from various organs including the brain. The ages at death are symmetrically distributed around the median duration of disease i s months. Most patients (76%) died within six months of onset. Retrosp ectively, 86% of patients fulfilled clinical criteria of probable or p ossible CJD. Neuropathology showed the classic triad of spongiform cha nge, astrogliosis, and neuronal loss in most cases. Two cases did not show unequivocal tissue alterations, but anti-PrP immunocytochemistry detected PrP deposits also in these cases. It is concluded that the re cent rise in incidence of CJD in Austria most likely reflects increase d awareness and diagnosis of CJD rather than a real increase. As bovin e spongiform encephalopathy (BSE) has not been reported in Austria, th e data do not support a link between a rise in incidence of sporadic C JD and BSE.