Abstract： Background Creutzfeldt-Jakob disease (CJD), a rare disease, is uncharacterized by computed tomography (CT)and magnetic resonance imaging (MRI). This study was aimed to evaluate the diffusion-weighted MRI (DWI)manifestations of CJD and to discuss their diagnostic value.Methods The findings of T1-weighted MRI (T1WI), T2-weighted MRI (T2WI), DWI and post-contrast MRI in 5patients (3 patients with biopsy-proven CJD and 2 patients with clinically-proven CJD) were retrospectivelyanalyzed in this study.Results Four out of the 5 patients had cerebral atrophy of various degrees. One patient showed symmetric highsignal intensity at the bilateral globus pallidus and the head of the caudate nucleus, with very high signal in thecerebral cortex on the DWI. This patient only had symmetric slightly high signal at the bilateral globus pallidusand putamen on T2WI. One patient had high signal intensity at the basal ganglia and cerebral cortex on DWI, butabnormal T2 signal intensity at the bilateral paraventricular white matter on MRI. Two patients presented withwidely gyri-like high signal intensity at the cortex on DWI, but routine MRI showed bilateral paraventricularlong T2 signal intensity in 1 patient and no abnormal findings in another. No abnormalities were shown by bothroutine MRI and DWI in the last patient.Conclusions DWI is more sensitive than its conventional counterpart in the depiction of CJD. DWI is moresensitive to detect cortical abnormal signal intensity in CJD not detected by T2WI.