进行性核上性麻痹的临床表现和神经影像学特点

Clinical and neuroimaging characteristics of progressive supranuclear palsy

目的探讨进行性核上性麻痹(progressive supranuclear palsy,PSP)的临床特点及头颅MRI、正电子发射体层扫描(PET)检查在本病中的诊断价值。方法回顾性分析19例PSP患者临床特点、神经影像学特征。结果19例PSP患者中,12例患者以走路不稳、反复向后跌倒为首发.17例患音出现垂直性核上性眼肌麻痹,假性球麻痹出现较早,还伴有轴性肌张力障碍、轻度痴呆等症状。19例患者均行头颅MRI检查,10例患者正中矢状位可见中脑上端萎缩,呈"蜂鸟征",水平位可见中脑前后径变小,呈"鼠耳征",1例患者中脑被盖和顶盖部T_2加权像显示弥散性高信号,中脑萎缩随病程加重。13例患者PET检查,均可见中脑葡萄糖代谢降低,双侧额叶葡萄糖代谢降低比较明显,部分合并顶叶或顶枕联合区葡萄糖代谢降低。结论 PSP临床表现变异较大,但通过头颅MRI、PET等辅助检查的特征性表现,可为诊断及鉴别诊断提供依据。

Objective To explore the clinical features and value of MR1 and ^18F-FDG PET in diagnosis of progressive supranuclear palsy（PSP）. Methods Retrospective analysis was conducted on clinical and neuroimaging features of 19 patients with clinically diagnosed probable PSP. Results The initial symptoms of PSP were walking instability and falls. Characteristically, supranuclear gaze deficits in PSP involved initially either downward or upward gaze. False bulbar palsy could be observed early, accompanied by axial dystonia and mild dementia symptoms. Cranial MRI scan was applied in all 19 patients. On mid-sagittal plane MRI,there were atrophy in upper part of the mid- brain and hummingbird sign. On horizontal plane MRI, reduction of anteroposterior diameter of midbrain and mouse ears sign were observed. Hyperintense signal was found on T2 WI MR image of the midbrain tegmentum and tectum of tients,and showed that glucose metabolism presented reduction of glucose metabolism one patient. 18 F-FDG PET was performed in 13 pa- in midbrain of all patients was low,some of them also in bilateral frontal and parietal lobes. Conclusion Some clinical characteristics of PSP are special, some auxiliary examinations, such as MRI and PET, are important for making the diagnosis and differential diagnosis.