原发性进行性失语的临床、影像及语言特征

Clinical, imaging, and linguistic profiles of primary progressive aphasia

目的 探讨原发性进行性失语（PPA）的临床、影像及语言特征.方法 PPA患者7例,其中语义性痴呆（SD）6例,进行性非流利性失语（PNFA）1例,收集患者的人口学资料、病史,进行头MRI检查,采用汉语失语成套测验进行语言评估.结果 患者平均发病年龄56岁,均缓慢起病,以语言表达、命名障碍为首发症状.MRI示左侧颞极萎缩为主,病程长的患者左侧额叶和顶叶、右侧颞叶也明显萎缩.语言评估发现所有患者的自发语言、复述、命名、听理解、阅读和书写均不同程度损害.SD患者言语流利,复述、朗读能力下降相对较轻,命名、复杂语句的理解能力损害突出.PNFA言语顿挫吃力,患者列名能力明显下降,但命名相对保存完好.结论 PPA多为老年前期发病,语言障碍为最早、最突出的症状.MRI特征性的改变为额叶和颞极萎缩,左侧为著.其中SD表现为命名性失语和经皮质感觉性失语,PNFA表现为经皮质运动性失语的特征.

Objective To explore the clinical, imaging, and linguistic features of the primary pro- gressive aphasia （PPA）. Methods Detailed demographic data and medical history were collected from 7 PPA patients, including 6 cases with semantic dementia （SD） and 1 patient with progressive nonfluent a phasia （PNFA）. Brain MRI scans and systemic linguistic evaluation were administered to all patients. Results The mean age at onset was 56 years. All patients developed slowly progressive word--finding difficulty and anomic speech. Atrophy, typically involving the left anterior temporal was shown on MRI scans in SD patients. In case with long course, there existed significant atrophy in left frontal lobe, pari etal lobe, and right temporal lobe. Language assessment found that all patients were impaired in sponta- neous expression, repetition, naming, auditory comprehension, reading and writing to varying degrees. SD patients had fluent language, but performed more poorly in naming, auditory comprehension, and writing. Having labored language, PNFA patient declined mostly in category verbal fluency, whereas naming ability was quite normal. Conclusions Current study suggested that PPA tend to develop in pre- senile age. Deficit in language is the earliest and most prominent symptom. Atrophy involving frontal lobe and temporal pole is characteristic change in MRI. The linguistic profile in SD is similar to that of name aphasia and transcortical sensory aphasia, while PNFA resemble the transcortical motor aphasia.