关键词: 克-亚综合征, 痴呆, 弥散磁共振成像

Abstract: Objective  To analyze the characteristics of  clinical manifestation and imaging in sporadic CreutzfeldtJakob disease.Methods  Clinical data of 37 patients with sporadic CreutzfeldtJakob  disease were retrospectively analyzed.Results  19 male and 18 female patients were included．The mean age at onset of male patients was 56.8 years old，while that at onset of female patients was 60.3 years old.17 patients firstly presented with impaired cognition. All of them presented with rapidly progressive dementia. 24 cases were with myoclonus．1 patient was in  akinetic mutism state. 17 cases showed  periodic sharpwave complexes on electroencephalogram， 22 patients showed 14-3-3 protein   in cerebrospinal fluid positive. 35 cases were hyperintensity of  frontal lobe， temporal lobe， parietal lobe and occipital lobe on diffusionweighted magnetic resonance imaging(DWI). Conclusion  Sporadic CreutzfeldtJakob disease mainly manifested with rapidly progressive dementia  and myoclonus. Diffusionweighted magnetic resonance imaging has high sensitivity and specificity for diagnosis of sporadic CreutzfeldtJakob disease.

Key words: CreutzfeldtJakob syndrome, dementia, diffusion magnetic resonance imaging