Author/Editor     Popović, Mara; Glavač, Damjan; Smerkolj, Sava; Švigelj, Viktor; Kalan, Gregor; Galvani, Vesna; Černilec, Maja; Bresjanac, Maja
Title     Creutzfeldt-Jakob disease in Slovenia from 1985 to 2003
Type     članek
Source     Wien Klin Wochenschr
Vol. and No.     Letnik 116, št. 15-16
Publication year     2004
Volume     str. 524-9
Language     eng
Abstract     Aim: The epidemic of bovine spongiform encephalopathy and subsequent emergence of a new variant of Creutzfeldt-Jakob disease have raised great public concern, initiating improved and prospective surveillance of human prion diseases in Europe and all over the world. This report briefly presents the epidemiology, clinical data, neuropathology, immunohistochemistry, biochemistry, and prion-protein gene analysis of Slovenian cases of Creutzfeldt-Jakob disease from January 1985 to the end of 2003. Materials, methods and results: During the 19-year period, 39 suspected cases of Creutzfeldt-Jakob disease were referred and 22 were confirmed. The prion-protein gene was analyzed in 12 of the confirmed cases and the protein glycosylation pattern in 11. There was a low average incidence of Creutzfeldt-Jakob disease (0.5/million) throughout the surveillance period, but a pronounced increase between January 2001 and December 2003 (to 1.9/million/year). A high female to male ratio (2.5/1) was noted. All of the confirmed cases were defined as sporadic Creutzfeldt-Jakob disease based on the clinical data, neuropathological findings, glycosylation pattern, and gene analysis. All tested cases had a type-2 glycosylation pattern; eleven of the twelve tested patients were homozygous at codon 129 of the prionprotein gene (1 VV and 10 MM) and one was heterozygous. Conclusion: The small number of Slovenian cases of sporadic Creutzfeldt-Jakob disease during the last 19 years has shown a pronounced increase in incidence, reflecting improved surveillance, and a high female to male ratio, where female cases are more than twice as numerous as male cases.
Descriptors     CREUTZFELDT-JAKOB SYNDROME
PRIONS
BRAIN
AGE OF ONSET
GLYCOSYLATION
AUTOPSY
INCIDENCE
IMMUNOHISTOCHEMISTRY
ELECTROENCEPHALOGRAPHY
POLYMORPHISM, SINGLE-STRANDED CONFORMATIONAL
BLOTTING, WESTERN