摘要目的 分析原发性进行性失语(PPA)患者的临床及影像学特点.方法 对天津市环湖医院2011年4月至2014年10月初诊,经随访临床诊断的进行性非流利型失语(PNFA) 15例(23％)、语义性痴呆(SD)16例(13％)、logopenic型进行性失语(LPA)1例患者的临床、神经心理量表及影像特点进行分析.结果 患者发病年龄40～69(59.6±7.0)岁,发病年龄＜65岁24例(75％),男女比例17∶15,有阳性家族史7例(22％).SD患者平均发病年龄较PNFA患者轻,性别上差异无统计学意义.波士顿命名及语言流畅性检测有很高的敏感度,SD患者较PNFA患者得分更低.其中23例患者伴有记忆力下降.SD患者MRI表现主要为双颞叶萎缩、左颞叶萎缩,PFNA患者MRI主要表现为左额颞、双额颞萎缩.5例患者头颅MRI显示无明显萎缩,核素18氟标记的2-(氟-18)-2-脱氧葡萄糖-正电子发射体层成像显示3例PNFA患者中1例表现为左额、左丘脑、左前扣带回代谢减低;2例表现为双额岛、尾状核丘脑、前扣带回、左颞代谢减低.2例SD患者中1例左颞、左尾状核代谢减低;1例双颞、左额、左岛、左枕代谢减低,左颞为著.1例LPA患者右颞顶、右岛及左顶代谢减低.LPA患者显示皮质淀粉样蛋白沉积.结论 PPA患者发病年龄较轻,多数患者伴有记忆力下降,脑萎缩及代谢减低主要在左额颞叶.LPA患者显示有淀粉样蛋白沉积.

abstractsObjective To identify the clinical profile and the neuroimaging characteristics of patients with primary progressive aphasia (PPA).Methods Of 32 patients diagnosed as PPA in the Memory Clinic of Tianjin Huanhu Hospital between April 2011 and October 2014,16 patients with semantic dementia (SD),15 patients with progressive non-fluent aphasia (PNFA),1 patient with logopenic progressive aphasia (LPA) were identified.Clinical data of the patients were collected.Cognitive function was measured by the Mini-Mental State Examination,Montreal Cognitive Assessment,Clinical Dementia Rating and Boston naming test.Brain magnetic resonance imaging was conducted to evaluate the cortex atrophy and medial temporal lobe atrophy.The 18F-fluoro-2-deoxy-D-glucose positron emission tomography (18F-FDG-PET) and Pittsburg Compound B (PiB) PET cerebral imaging were performed in patients with atypical clinical and MRI demonstrations.Results The onset age was 40 to 69 (59.6 ± 7.0) years,and 24 patients (75％) were younger than 65 years.The ratio of male to female was 17 to 15.Seven patients (22％) have positive family history.The average onset age of SD patients was younger than PNFA patients.The Boston naming test and verbal fluency test had a high sensitivity in PPA patients,and the scores of SD patients were lower than PNFA patients.Twenty three of the patients had memory loss.Patients with SD showed bitemporal lobe atrophy,left temporal lobe atrophy.PFNA patients mainly manifested as left frontotemporal,bilateral frontotemporal atrophy on MRI scan.Five patients (3 PNFA,2 SD) with no obvious atrophy on head MRI had FDG-PET scan.Among the three PNFA patients,one showedhypometabolism on the left frontal,left thalamus,left anterior cingulate,and two showed hypometabolism on bilateral frontal,insula,thalamus,caudate nucleus,anterior cingulate,and left temporal lobe.Among the two SD patients,one showed left temporal,left caudate nucleus hypometabolism,and one showed bilateral temporal (left ＞ right),left frontal,left insula,left occipital hypometabolism.One LPA patient showed right temporal,parietal lobe,insula,and left parietal lobe hypometabolism.LPA patients showed cortical amyloid deposition.Conclusions PPA patients have younger onset age.The majority of patients have memory loss.Brain atrophy and hypometabolism are mainly on the left frontotemporal lobe.LPA patients show deposition of amyloid protein.