Creutzfeldt -Jakob disease as being the most common one among the other rare prion disesase, is characterised with progressive demans, myoclonic jerks and a variety of other visual and neurological abnormalities. Twelve patients with the diagnosis of Creutzfeldt-Jakob disease were evaluated, ten of whom retrospectively and the rest prospectively, according to the clinical, radiological and pathological findings. Diagnostic value of the clinical features is discussed in the view of the literature.