Author/Editor		Koren, Srečko
Title		Laboratorijska diagnostika prionskih bolezni
Type		članek
Source		In: Dragaš AZ, Fišer J, Prinčič D, et al, editors. Zbornik strokovnega srečanja Mikrobiološka analiza kužnin; 1999 okt 22-23; Nova Gorica. Nova Gorica: Zavod za zdravstveno varstvo,
Publication year		1999
Volume		str. 49-54
Language		slo
Abstract		Prion disease or transmissible spongiform encephalopathies are rare, fatal human and animal neurological diseases that are most probably caused by pathogenic cellular proteins, termed prions. The most important form of prion disease in humans is Creutzfeldt-Jacob disease (CJD). New variant CJD was first descibed in the UK in 1996 and is believed to be related to the consumption of beef from cattle infected with bovine prions... Classical CJD may be suspected on clinical grounds and EEG findings but definitive diagnosis of the disease can be made only by histopathologic examination of brain tissue. The cerebrospinal fluid of most patients with CJD contains abnormal proteins. New variant CJD has atypical clinical and neuropathological features. The diagnosis of new variant CJD can be made by Western blotting. It has been demonstrated that new variant CJD is associated with a specific pattern of prion protein fragment size on Western blot (glycosylation dependent) which distinguishes it from other forms of CJD. Recently rapid immunological and molecular tests which can detect prion proteins of new variant CJD in tonsil biopsies have been described.
Summary		Prionske bolezni in prenosljive spongiformne encefalopatije so redke, smrtne degenerativne bolezni osrednjega živčevja pri člobeku in živalih, ki jih povzročajo patogene celične beljakovine, imenovane prioni. Najpomembnejša prionska bolezen pri človeku je Creutzfeldt-Jakobova bolezen (CJB). Nova različica CJB je bila prvič opisana leta 1996 v Veliki Britaniji. Povezujejo jo z uživanjem hrane, okužene z govejimi prioni. Na klasično obliko CJB lahko posumimo na osnovi kliničnih znakov in izvidov EEG, za dokončno diagnozo pa je nujen histopatološki pregled možganskega tkiva. V cerebrospinalnem likvorju večine bolnikov s CJB lahko odkrijemo posebne beljakovine. Nova različica CJB največkrat poteka z neznačilnimi kliničnimi znaki in nevropatološkimi spremembami. Laboratorijsko dokazujemo novo različico CJB z metodo Western blot, s katero ugotavljamo glikozilacijski vzorec patogene prionske beljakovine v izvlečkih možganov. V novejšem času so opisali hitre imunske in molekulske teste za dokaz prionske beljakovine nove različice CJB v limfatičnem tkivu žrela.
Descriptors		PRION DISEASES CREUTZFELDT-JAKOB SYNDROME IMMUNOLOGIC TESTS