Background. Creutzfeldt-Jakob disease (CJD) is a difficult to diagnose prion disease, characterized by the development of rapidly progressive dementia and a long incubation period, which leads to death within the first year in 90% of cases. Despite the existence of criteria for intravital diagnosis and modern technologies, histotyping of the material still remains the gold standard for making a final diagnosis. The sporadic form remains the most common among all types of CJD.
Objective. Analysis of current data on CJA, systematization of the information obtained to facilitate the differential diagnosis of CJD types.
Methods. Data from 11 studies with a total of 817 confirmed cases of CJD were used for this work. The phenotypic features for each known type and subtype were analyzed, and the systematic sequence of distribution of the PrPsc prion protein for the two most common types of CJD was also indicated.
Results. Due to the studies reviewed, we are convinced that the true diversity of CJD histotypes is much wider than previously thought. Along with typical M1, M2C, M2T, VV1, and VV2 CJD forms, researchers distinguish transitional forms – VV1–VV2, and subtypes with specific morphological features – MV 1C–2PL.
Conclusion. The obtained data can be applied in practice if it is necessary to differentiate the types of CJD

Keywords: neurodegenerative disease, Creutzfeldt-Jakob disease, prions, phenotype, dementia