Abstract: Objective: To investigate the early clinical diagnosis of Creutzfeldt-Jakob disease(CJD).Methods: The clinical manifestation,electroencephalogram(EEG) and imaging data of two cases of CJD were analyzed retrospectively.Results: There were special clinical characteristics of CJD in Chinese.The characteristic lesions were detected by diffusion-weighted imaging(DWI) in both of the two cases.DWI was more sensitive than the current clinical diagnosis method suggested by WHO.Conclusions: Recognition of the clinical characteristics of CJD in Chinese is helpful to early and correct diagnosis of the disease and can reduce the iatrogenic transmission.DWI,a non-invasive diagnostic method,is the most sensitive test for the early diagnosis of CJD and consideration should be given to its inclusion in the clinical diagnostic criteria of CJD.