: Creutzfeldt-Jakob disease (CJD) is a subacute spongiform encephalopathy. This fatal prion disease is characterized by rapidly progressive dementia with a variety of neurological disorders. Diagnostic methods provided by nuclear medicine might be helpful for evaluation of patients with probable CJD as additional diagnostic tools to MRI and cerebro-spinal fluid evaluation. The experience with FDG-PET and brain perfusion SPECT is presented.