florzolotau(^(18)F)正电子发射体层摄影显像协助诊断的进行性核上性麻痹小脑型3例并文献复习

Florzolotau(^(18)F)positron emission tomography imaging assisted diagnosis of progressive supranuclear palsy with predominant cerebellar ataxia:3 cases report and literature review

目的总结3例florzolotau(^(18)F)正电子发射体层摄影(tau PET)显像协助诊断的进行性核上性麻痹小脑型(PSP-C)患者的临床表现、结构和功能影像学特征,并进行相关文献回顾,旨在为PSP-C这一少见类型诊治提供更多参考依据。方法总结2019年1月至2021年12月就诊于北京协和医院神经科的3例PSP-C患者的临床资料、头颅磁共振成像、头颅18氟-氟代脱氧葡萄糖PET(^(18)F-FDG PET)和tau PET影像特征,同时对国内外相关病例报道或系列研究进行系统性回顾分析。结果3例患者发病年龄55~61岁,至就诊时病程2~5年,均以行走不稳起病,并出现反复多次跌倒,其中跌倒距离发病0.5~3.0年,平均1.5年。诊断时患者均存在步态共济失调,伴或不伴肢体共济失调。帕金森专项磁共振结果显示所有患者均存在中脑萎缩,中脑/脑桥比值均<0.52。所有患者的tau PET结果均显示中脑显著tau蛋白沉积、小脑轻至中度tau蛋白沉积,例2同时存在前额叶轻度tau蛋白沉积且该区域^(18)F-FDG PET代谢减低,支持PSP诊断。经复习文献,既往报道PSP-C患者24例,其中提供详细临床资料者23例。对这些患者的临床和影像特征进行的总结结果表明,本组患者的特点与既往报道的病例基本一致。结论PSP-C以早期步态共济失调和跌倒为核心表现,结构影像显示中脑萎缩,tau PET显示中脑、小脑摄取。对于非经典型PSP,头颅磁共振成像联合tau PET成像有助于进一步确定PSP的诊断。

ObjectiveTo report the clinical manifestations,structural and functional imaging features of 3 patients with progressive supranuclear palsy with predominant cerebellar ataxia(PSP-C)assisted by florzolotau(^(18)F)positron emission tomography(tau PET)imaging,and conduct a literature review,aiming to provide a basis for the diagnosis and treatment of this rare type of PSP.MethodsThe clinical data,brain magnetic resonance imaging,^(18)F-fluorodeoxyglucose PET(^(18)F-FDG PET)and tau PET head imaging features of 3 patients with PSP-C who were admitted to the Department of Neurology,Peking Union Medical College Hospital from January 2019 to December 2021 were summarized,and a systematic review of related case reports or series studies from China and abroad was conducted.ResultsThe age of onset of the 3 patients was 55-61 years,and the disease duration was 2-5 years at the time of diagnosis.All patients had an onset of instable walking and had repeated falls,and the duration between fall and disease onset was 0.5-3.0 years,with an average of 1.5 years.At the time of diagnosis,all patients showed gait ataxia with or without limb ataxia.The results of the brain magnetic resonance imaging showed that all patients had midbrain atrophy and midbrain-to-pons ratio<0.52.The tau PET results of all patients showed significant tau protein deposition in the midbrain and mild to moderate tau protein deposition in the cerebellum,and case 2 had concomitant mild tau protein deposition in the prefrontal lobe and decreased^(18)F-FDG PET metabolism in this region,supporting the diagnosis of PSP.Literature review showed that 24 patients with PSP complicated with cerebellar ataxia were reported,and 23 patients provided detailed clinical data.All patients had gait ataxia on physical examination and the clinical manifestations were consistent with those of this group.ConclusionsPSP-C is characterized by early gait ataxia and falls as the core manifestations.Structural imaging shows mesencephalic atrophy,and tau PET shows mesencephalic and cerebellar uptake.In the case of atypical PSP,head magnetic resonance imaging combined with tau PET imaging is helpful to further determine the diagnosis of PSP.