摘要
目的总结克-雅病患者^(18)F-脱氧葡萄糖(^(18)F-FDG)PET影像学特点。方法与结果共纳入2018年8月至2020年1月8例克-雅病患者(包括散发性克-雅病7例、遗传性克-雅病1例),临床主要表现为快速进展性痴呆。DWI均存在皮质和(或)基底节区高信号(8/8例);^(18)F-FDG PET均呈单侧或双侧额顶颞叶葡萄糖低代谢(8/8例),其次依次为枕叶(7/8例)、丘脑(5/8例)、基底节区(3/8例)、脑干(3/8例)和小脑(3/8例)低代谢。对比分析葡萄糖低代谢区域与相应DWI高信号区域,发现皮质50个葡萄糖低代谢区域中40个呈DWI高信号,尤以额叶的一致性较好;皮质下25个葡萄糖低代谢区域中仅9个呈DWI高信号,尤以基底节区的一致性较好;脑干和小脑仅呈现葡萄糖低代谢。结论克-雅病的诊断与鉴别诊断具有较高的敏感性,特别是病情进展迅速的患者,^(18)F-FDG PET低代谢多提示病变部位神经功能严重缺损。对于快速进展性痴呆且伴明显锥体外系症状或小脑症状的患者,^(18)F-FDG PET显示皮质低代谢而DWI未见明显高信号,应考虑克-雅病的诊断。
Objective To explore the characteristics of^(18)F-fluoro-2-deoxy-D-glucose (^(18)F-FDG)PET in Creutzfeldt-Jakob disease(CJD).Methods and Results confirmed as CJD were analyzed retrospectively from August 2018 to January 2020. Five patients were male and 3 were female, with a mean age of(49.63 ± 13.19) years. There were 7 sporadic CJD(sCJD)patients and one genetic CJD(gCJD) patient. All 8 patients were characterized by rapid progressive dementia. DWI hyperintensity in brain MRI were observed in all 8 patients.^(18)F-FDG PET analysis revealed frontal, parietal and temporal regions hypometabolism in all 8 cases. Occipital region hypometabolism was observed in 7 cases(7/8). For subcortical regions, hypometabolism were identified in thalamus(5/8), basal ganglia(3/8), brainstem(3/8) and cerebellum(3/8). Compared with the DWI hyperintensities, there were 40 hyperintense on DWI in the 50 cortical regions that were hypometabolic on^(18)F-FDG PET, especially in the frontal lobe. For subcortical regions, there were only 9 hyperintense on DWI in the 25 regions that were hypometabolic on^(18)F-FDG PET, especially the basal ganglia region. The brain stem and cerebellum hypometabolic regions showed no hyperintense on DWI.Conclusions with rapid progression.^(18)F-FDG PET hypometabolism may indicate severe focal neurological impairment.The diagnosis of CJD should be considered for patients with rapidly progressive dementia accompanied with obvious symptoms of extra pyramidal system or cerebellum, cortical hypometabolism in^(18)F-FDG PET, and without obvious DWI hyperintensity.
作者
林雪
甄祯
易芳
杨吉刚
关鸿志
石琦
刘磊
王佳伟
郭燕军
LIN Xue;ZHEN Zhen;YI Fang;YANG Ji-gang;GUAN Hong-zhi;SHI Qi;LIU Lei;WANG Jia-wei;GUO Yan-jun(Department of Neurology,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China;Department of Neurology,PLA Rocket Force General Hospital,Beijing 100088,China;Department of Nuclear Medicine,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China;Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China;National Institute for Viral Disease Control and Prevention,Chinese Center for Disease Control and Prevention,Beijing 102206,China;Medical Research Center,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China)
出处
《中国现代神经疾病杂志》
CAS
北大核心
2021年第5期371-377,共7页
Chinese Journal of Contemporary Neurology and Neurosurgery
基金
国家自然科学基金青年科学基金资助项目(项目编号:81301032)
传染病预防控制国家重点实验室开放课题(项目编号:2020SKLID311)。
