Jozef Haring ml., Georgi Krastev
Creutzfeldt - Jacob disease (CJD) is fatal progressive neurodegenerative disease, belonging to the group of transmissible human spongiform encephalopathies. It is caused by multiplication and cumulation of pathologic prion proteins PrPres in human brain. Sporadic CJD is the most common form, its etiology is unknown, and incidence is approximately 1 to 1.5 cases in million people. Clinical symptoms are various, imitating other diseases. Patients between 45 and 75 years are affected; men and women are affected in the same ratio, leading to death in 6 months. In retrospective analysis of 17 patients with suspicion of CJD between 2002 and 2006 we decided to apply Poser,s diagnostic criteria, including protein 14-3-3 and typical MRI findings. We tried to investigate how these additional criteria are specific for our group of patients as a diagnostic marker of sCJD already during life. We found out high specificity of typical MRI findings – hyperintensive signal of nc. caudatus and putamen and also some cortical structures of brain and sensitivity of protein 14-3-3, in correlation with already published studies.