中文摘要：

目的 比较原发性进行性失语logopenic型和非流利型两种临床亚型认知和言语障碍的差异,分析言语和认知障碍对日常生活能力下降的不同影响.方法 首都医科大学宣武医院神经内科记忆门诊2015年1月至2016年1月共纳入logopenic型原发性进行性失语（lv-PPA）7例,非流利型原发性进行性失语（nfv-PPA）5例,年龄匹配的健康对照者20名.所有患者和对照者均完成MMSE、CDR、ADL,成套语言量表、头颅核磁.所有患者完成11C-PiB-PET检查.结果 lv-PPA患者言语障碍为词语提取障碍、命名和复述障碍,而nfvPPA表现为突出的发音失用和发音失真.lv-PPA发病年龄[（56±5）岁]低于nfv-PPA[（61±5）岁],差异有统计学意义（P＜0.05）.大部分Iv-PPA患者病程在1.5年内进展到痴呆水平,日常生活能力显著受损,伴有多项顶叶皮质功能障碍如观念运动性失用、Gerstmann综合征和结构性失用.4例nv-PPA患者在2年病程内仅有轻度认知功能障碍,未见顶叶皮质功能障碍,日常生活能力保存较好.与言语障碍相比,顶叶皮质功能障碍与整体认知功能障碍、日常生活能力下降具有更强的相关性（r=-0.868,r=-0.922）.11C-PiB-PET检查7例lv-PPA均为阳性,5例nfv-PPA均为阴性.结论 相比nv-PPA,lv-PPA患者认知障碍和顶叶皮质障碍更为显著,病情进展更快.与言语障碍相比,皮质功能障碍和认知功能障碍对日常生活能力的影响更强.

英文摘要：

Objective To decipher the cognitive and linguistic feature of logopenic variant primary progressive aphasia （lv-PPA） and nonfluent variant primary progressive aphasia （nfv-PPA） and to explore the extent to which cognitive and language impairment contribute to the dysfunction of activity of daily living （ADL）.Methods Seven lv-PPA and five nfv-PPA were enrolled in memory clinic of Xuanwu Hospital,Capital Medical University from January 2015 to January 2016 accordig to the international consensus criteria for PPA and its three subtypes.20 age-matched normal controls （NC） were included.Both the patients and the NC completed a battery of neuropsychological test,lingusitic test and brain magnetic resonance imaging.All the patients conducted 11C Pittsburgh compound B （PiB） PET imaging.Result Lv-PPA patients were characterized by deficits in lexical retrieval and long sentenses repetition,while nfv-PPA were with motor speech apraxia and phonetic distortion.Compared with nfv-PPA,lv-PPA patient displayed more severe cognitive deficit with younger onset of age （56 ± 5 vs 61 ± 5,P 〈 0.05）,rapid decline of MMSE score within 1.5 years and pariental cortex dysfunctions such as ideomotor praxis,Gerstmann syndrome and contructional apraxia.Correlation analysis indicated that there was more significant association between pariental cortex dysfunction and ADL/mini-mental state examination （MMSE） than that of language deficit （r =-0.868,r =-0.922;r =0.312,r =-0.257）.All seven lv-PPA were PiB-PET positive and five nfv-PPA were negative.Conclusion This study enriched the chinical and linguistic characterization of lvPPA and nfv-PPA,which has implication for diagnosis,disease management and treatment for clinicians.