Tehran University of Medical Sciences Acta Medica Iranica 0044-6025 52 6 2014 06 15 488 489 EN Payam Sarraf Department of Neurology, Faculty of Medicine, Tehran University of Medical Sciences, Tehran, Iran. Mahsa Ghajarzadeh Department of Neurology, Brain and Spinal Injury Research Center, Tehran University of Medical Sciences, Tehran, Iran. Babak Salarian Department of Neurology, Tehran University of Medical Sciences, Tehran, Iran. 2015 10 11 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. https://acta.tums.ac.ir/index.php/acta/article/view/4773 https://acta.tums.ac.ir/index.php/acta/article/download/4773/4475