Abstract: Creutzfeldt-Jacob Disease is the most frequently seen type of prion diseases. Its clinical findings consist of predominantly progressive dementia with a rapid onset, myoclonus, and also cerebellar, pyramidal, extrapyramidal and visual signs. Occurrence of periodical spikes in EEG, observation of cortical signal alterations during diffusion weighted (DW) MRI studies, and detection of protein 14-3-3 in cerebrospinal fluid (CSF) substantiate the diagnosis. Definitive diagnosis is established with histological examination of brain biopsy or autopsy materials.