The problem list which led to final confirmation of the disease included non-specific neuropsychological presentations, hypersignality in caudate and putamen areas in brain MRI, generalized high frequency sharp waves in EEG, and protein 14-3-3 identification in cerebrospinal fluid.<h4>Conclusion</h4>Although CJD is not a common disease, it should be considered in differential diagnoses whenever neuropsychological manifestations, especially progressive decline in cognition, along with symptoms such as visual hallucinations, myoclonus and ataxia are observed in the patient. This evidence concerns the gene YWHAQ and Creutzfeldt Jacob disease.