ObjectiveTo explore the pathogenesis，etiological subtype，clinical manifestation，cerebrospinal fluid (CSF) characters，magnetic resonance imaging(MRI) findings，electroencephalogram （EEG)  changes and diagnosis criteria of CreutzfeldtJacob disease(CJD).MethodsAnalysis was made in the clinical data of two CJD patients from the hospital. ResultsCJD showed the clinical manifestation predominantly of progressive dementia，ataxia and myoclonus. Occurrence of periodic sharp wave complexes(PSWCs) in EEG， hyper intense signal changes in cortical regions (cortical ribboning)  in MRI， and protein 1433 in CFS substantiate all supported the diagnosis. ConclusionCJD has typical characters of EEG，MRI and CFS. Specially， diffusion weighted imaging may be a noninvasive screening tool with higher sensitivity and specificity.