ABSTRACT
Creutzfeldt-Jakob disease [C-JD] is a rare disorder characterized with rapidly progressive mental decline, myoclonic jerk and finally death. The transmissible pathogen for this disease is a proteinaceous infectious particle termed prion. The prion protein is encoded by a gene [designated as PRNP] on the short arm chromosome 20.This disorder is diagnosed based on clinical findings, course of disease, EEG, MRI and confirmed with brain biopsy. A 56- year- old woman presented with confusion, disorientation, hyper somnolence, psychiatric problems such as hallucination, progressive mental deterioration and myoclonic jerks. She had history of several times phlebotomy with traditional and non sterile methods in two past years. She had no past history of other disease. Her illness was diagnosed based on clinical findings, course of her illness, typical MRI, EEG and rule out other dementing disease. She died after one month. in any patients with psychiatric disorders, rapidly progressive mental deterioration and myoclonic jerks C-JD should be considered as an important diagnosis. Treatable dementing disease should be considered and ruled out at first. The significance of phlebotomy in C-JD has yet to be determined