Author/Editor		Popović, M; Glavač, D; Smerkolj, S; Švigelj, V; Kalan, G; Bresjanac, M
Title		Human prion diseases in Slovenia 1985 to 2003
Type		članek
Source		In: Vizjak A, Ferluga D, Bussolati G, editors. Update in pathology. Proceedings of the 19th European congress of pathology: nephropathology pre-congress meeting advances in nephrology, pulmonary pathology pre-congress meeting; 2003 Sep 6-11; Ljubljana. Ljubljana: Faculty of medicine,
Publication year		2003
Volume		str. 311-3
Language		eng
Abstract		The epidemic of bovine spongiform encephalopathy (BSE) and subsequent emergence of new variant of Creutzfeldt-Jakob disease (vCJD) have raised a great public concern, initiating active surveillance on human prion diseases in Europe and all over the world. Report briefly presents Slovenian CJD cases from January 1985 through June 2003 regarding epidemiology, clinical data, neuropathology, immunohistochemistry and PRNP gene analysis. Thirty six suspected CJD cases were referred for evaluation, and twenty one were confirmed. PRNP was analyzed in twelve of the confirmed cases. There are two outstanding characteristics of the reported Slovenian CJD cases: (1) a low average incidence of CJD (0.5/million) throughout the surveillance period, with a pronounced increase from January 2001 through June 2003 (to 2/million/year), and (2) high female to male ratio (2.5/1). All our confirmed cases were defmed as sporadic CJD (sCJD) based on the neuropathological findings, clinical data, and gene analysis. Eleven of twelve tested patients were homozygous at codon 129 of the PRNP (1 VV and 10 MM) and one was heterozygous. The small number of Slovenian sCJD cases revealed a pronounced increase in incidence achieved through the active surveillance, and high female to male ratio, where females represent more than twice the number of male cases.
Descriptors		PRION DISEASES SLOVENIA