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Creutzfeldt-Jacob Disease is a prion disease which has a wide range of clinical presentations. Its diagnosis is not simple and clinical manifestation along with EEG, MR imaging findings and cerebrospinal fluid (CSF) analysis should be considered for a definite diagnosis. A-50-year-old woman referred with cognitive impairment, myoclonic jerks, mutism and difficulty in swallowing to our clinic. EEG (Electroencephalography) results showed bilaterally periodic sharp and slow-wave discharges. Protein 14-3-3 in CSF was detected. Magnetic resonance imaging (MRI) findings revealed hyperintensity of the caudate and putamen in diffusion-weighted imaging (DWI), T2 Weighted (T2W) sequences and Fluid-attenuated inversion-recovery (FLAIR) images. Patients who have progressive dementia should be evaluated by means of MR imaging and CSF analysis for CJD specific proteins should be considered.