致死性家族性失眠患者脑葡萄糖代谢分析

目的 研究致死性家族性失眠(fatal familial insomnia,FFI)患者脑葡萄糖代谢变化特征.方法 对病程分别为2个月的患者1和6个月的患者2以及20名健康对照者进行18F-脱氧葡萄糖(18F-fluorodeoxyglucose,18F-FDG)PET静态显像.采用视觉分析的方法判断2例患者脑代谢改变情况,然后利用统计参数图分析方法对每例患者和与其年龄相匹配的10名健康对照者进行组间分析,判断其代谢改变特征.结果 与10名年龄相匹配的健康对照组相比,患者1表现为明显的丘脑、顶叶、尾状核、后扣带回和前额叶的代谢减低(t＞2.82,P＜0.01).患者2表现为明显的丘脑、顶叶、后扣带回和前额叶的代谢减低,其代谢减低的范围和程度明显大于患者1(t＞2.82,P＜0.01),并伴有颞叶和枕叶代谢减低(t＞2.82,P＜0.01).结论 FFI患者脑葡萄糖代谢改变主要为双侧丘脑和大脑皮质代谢减低,大脑皮质所累及的范围和程度随病程发展而增大.18F-FDG PET显像对FFI的诊断和鉴别诊断具有一定的参考价值.

Brain metabolic analysis of fatal familial insomnia

Objective To investigate the characteristics of regional cerebral glucose metabolism in patients with fatal familial insomnia(FFI) using 18F-fluorodeoxyglucose(18F-FDG) PET. Methods Patient 1 with symptoms for 2 months and patient 2 with symptoms for 6 months were studied by brain 18 F-FDG PET.Compared with 20 normal controls, the data were analyzed by visual analysis at first, and then each patient was compared with age-matched normal controls using statistical parametric mapping( SPM ). Results As compared with 10 normal controls, metabolic changes in patient 1 was characterized by hypometabolism in thalamus, parietal cortices, caudate nucleus, pre-frontal cortices and posterior cingulate gyrus ( t ＞ 2. 82,P ＜0. 01 ). In patient 2, these changes were more obvious (t ＞ 2. 82, P ＜ 0. 01 ) with metabolic decrease also shown in temporal and occipital cortices ( t ＞ 2. 82, P ＜ 0. 01 ). Conclusion In FFI patients, brain metabolism changes are mainly manifested as hypometabolism in thalamus and cerebral cortex. The metabolic changes in cerebral cortex will be more widely spread with the development of FFI. 18F-FDG PET imaging was a valuable method to evaluate patients with FFI.