皮质基底节综合征患者临床及影像学特点分析  

Characteristics of clinical and neuroimage findings in patients with corticobasal syndrome

作　　者：许春燕 陈淑芬 孙云闯[2] 陈科良 葛璟洁 左传涛 崔梅 董强 郁金泰 Xu Chunyan;Chen Shufen;Sun Yunchuang;Chen Keliang;Ge Jingjie;Zuo Chuantao;Cui Mei;Dong Qiang;Yu Jintai(Department of Neurology,Huashan Hospital,Fudan University,Shanghai 200040,China;department of Neurology,Peking University First Hospital,Beijing 100034,China;Positron Emission Tomography Center,Huashan Hospital,Fudan University,Shanghai 200040,China;Department of Neurology,Luohu People’s Hospital,Shenzhen 518000,China)

机构地区：[1]复旦大学附属华山医院神经内科,上海200040 [2]北京大学第一医院神经内科,北京100034 [3]复旦大学附属华山医院PET中心,上海200040 [4]深圳市罗湖区人民医院神经内科,深圳518000

出　　处：《中华神经科杂志》2022年第6期626-633,共8页Chinese Journal of Neurology

基　　金：国家自然科学基金(81971641,81300997);上海市卫生健康委老龄化和妇儿健康研究专项(2020YJZX0111);上海申康医院发展中心临床三年行动计划(SHDC2020CR1038B);深圳市医疗卫生三名工程项目(SZSM201801014)。

摘　　要：目的探讨皮质基底节综合征(CBS)患者的临床表现、神经心理、头颅磁共振成像(MRI)、多模态正电子发射体层摄影(PET)/CT特点及CBS的病因诊断。方法回顾性分析2019年4月至2021年7月在复旦大学附属华山医院神经内科认知障碍病区收治的12例临床诊断为CBS患者的临床表现、神经心理学评估结果、头颅MRI及PET/CT影像学资料,并根据阿尔茨海默病、进行性核上性麻痹及皮质基底节变性等疾病的诊断标准等对12例患者进行病因学诊断。结果12例CBS患者均出现高级皮质功能障碍及不对称性运动障碍,对左旋多巴反应差;均有不同程度的认知功能受损[简易精神状态检查量表评分(16.16±9.82)分,蒙特利尔认知评估量表评分(13.44±7.35)分]。头颅MRI均出现不对称的以中央前后回萎缩为主的额顶叶皮质萎缩,5例患者出现中脑萎缩。葡萄糖代谢PET检查均显示不对称的额叶及基底节区(以尾状核及壳核明显)低代谢(受累肢体对侧明显)。完成tau蛋白PET检查的11例患者均显示存在皮质和(或)皮质下的tau蛋白沉积;完成淀粉样蛋白PET检查的4例患者中有2例出现大脑皮质淀粉样蛋白沉积。结合CBS患者的临床、头颅MRI、PET/CT分子影像学结果及相应诊断标准,最终有6例患者被诊断为进行性核上性麻痹,1例诊断为皮质基底节变性,5例诊断为为阿尔茨海默病。结论CBS病因异质性较大,结合患者的临床表现、神经心理学评估、头颅MRI及PET/CT分子影像学结果有助于CBS疾病的诊断。Objective To investigate the clinical,neuropsychological,and neuroimage characteristics in patients with corticobasal syndrome(CBS),and to elucidate the exact diagnosis of CBS patients.Methods Twelve CBS cases admitted to the Department of Neurology,Huashan Hosiptal,Fudan University from April 2019 to July 2021 were retrospectively enrolled in this study.Those data,including clinical features(demographic data and clinical characteristics of cortical dysfunction and movement disorder),neuropsychological assessment[Mini-Mental State Examination(MMSE)and Montreal Cognitive Assessment(MoCA)scales score],brain magnetic resonance imaging(MRI)and multi-mode positron emission tomography(PET)/CT,were collected and carefully reviewed.Exact diagnosis of these patients was given according to the disease diagnosis criteria.Results Cortical dysfunction and asymmetrical movement disorders were found in all cases,with poor response to levodopa.Patients suffered from cognitive impairment(MMSE score 16.16±9.82,MoCA score 13.44±7.35).The cranial MRI demonstrated significant asymmetric atrophy of frontal and parietal lobes,especially in the pre-and post-central gyrus.Fluorodeoxyglucose PET of 12 patients showed asymmetric frontal lobe and basal ganglia(especially caudate and putamen)hypometabolism(obviously on the contralateral side of the affected limb).Tau PET was implemented in 11 patients and displayed that abnormal tau protein deposition was positive in the cortex and/or subcortex in all patients.Of the 4 cases,who completed amyloid PET,amyloid protein deposition was positive in the cortex of 2 patients.As a result,6 patients were diagnosed as progressive supranuclear palsy,1 patient was diagnosed as corticobasal degeneration,and 5 patients were diagnosed as Alzheimer′s disease.Conclusions The etiology of CBS is heterogeneous.The combination of clinical manifestation,cranial MRI and multi-mode PET/CT helps the differential diagnosis of CBS.

关 键 词：皮质基底节综合征 皮质基底节变性 正电子发射断层显像技术 

分 类 号：R741[医药卫生—神经病学与精神病学]