以原发性进行性失语起病的皮质基底节变性1例分析  

Analysis of a case of corticobasal degeneration started with primary progressive aphasia

作　　者：黄森 林家宁 黄翩 陈伟能 郑民缨[1] 姚晓黎[1] Huang Sen;Lin Jianing;Huang Pian;Chen Weineng;Zheng Minying;Yao Xiaoli(Department of Neurology,the First Affiliated Hospital,Sun Yat-sen University,Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases,National Key Clinical Department and Key Discipline of Neurology,Guangzhou 510080,China)

机构地区：[1]中山大学附属第一医院神经科广东省重大神经疾病诊治研究重点实验室国家临床重点专科和国家重点学科,广州510080

出　　处：《中华神经科杂志》2022年第4期336-340,共5页Chinese Journal of Neurology

基　　金：国家重点研发计划项目(2017YFC0907703);国家自然科学基金青年科学基金(82001332);广东省重大神经疾病诊治研究重点实验室(2020B1212060017);广东省神经系统疾病临床医学研究中心(2020B1111170002);华南神经疾病早期干预及功能修复研究国际联合研究中心(2015B050501003,2020A0505020004);广东省神经系统重大疾病诊治工程技术研究中心;广东省神经系统重大疾病诊治转化医学创新平台;广州市神经系统重大疾病临床医学研究与转化中心(201604020010)。

摘　　要：目的:通过1例以原发性进行性失语(PPA)表现为首发症状的皮质基底节变性(CBD)的典型病例,探讨CBD的临床特征以及相关失语的特殊表现。方法:回顾性分析2020年7月中山大学附属第一医院收治的1例以PPA症状为主的CBD患者,总结其临床特点和诊断思路。结果:该患者为59岁男性,临床表现为快速进展的言语功能减退伴记忆力下降,失语特点为语速减慢、内容减少和语法错误,为PPA非流利语法变异表型,患者运动症状较轻,影像学检测提示左侧大脑皮质、基底节和丘脑萎缩并放射性摄取减低,最终诊断为可能CBD。结论:以PPA首发的CBD较为罕见,临床特点具有高度的非特异性,缺乏典型的运动症状会增加诊断的困难,及时的核医学功能成像以及生物学标志物检测有助于非典型CBD的早期诊断。Objective Through a typical case of corticobasal degeneration(CBD)with primary progressive aphasia(PPA)to analyze the clinical characteristics of CBD and the special manifestations of aphasia with that disease.Methods Retrospective analysis was performed on a patient with PPA based CBD who was admitted to the First Affiliated Hospital,Sun Yat-sen University in July 2020 to summarize the clinical features and diagnostic thinking of CBD.Results The patient was a 59-year-old male,manifested rapidly progressive dysfunction of language and memory function.The aphasia was mainly featured as slow speech,reduced content and grammatical errors,and diagnosed as PPA,non-fluent grammatical variation.The imaging results showed the atrophy of the left frontal lobe,parietal lobe,basal ganglia and thalamus,coupled with the reduction in 18F-fluorodeoxyglucose radioactive uptake.The patient was finally diagnosed as possible CBD.Conclusions PPA as the initial manifestation of CBD is very rare in clinical practice.The high non-specificity of clinical features and the lack of typical motor symptoms result in the difficulty of correct diagnosis of CBD.Timely functional imaging in nuclear medicine and reliable biomarkers help to facilitate early diagnosis of atypical CBD.

关 键 词：基底神经节 神经变性 失语 原发进行性 运动障碍 临床特点 

分 类 号：R741[医药卫生—神经病学与精神病学]