Objective To 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)posi...Objective To 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.Methods The 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.Results The 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.Conclusion PSP-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.展开更多
文摘Objective To 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.Methods The 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.Results The 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.Conclusion PSP-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.
