目的　原发性进行性失语 (PPA)是一种少见的中枢神经系统变性疾病 ,国内罕见报道。现报道 1例 ,以提高临床医生对该病的认识。方法　采用韦氏记忆量表、认知能力筛选检查、积木测验、数字广度测验和社会功能问卷等全套神经心理学量表方法 ,检查和描述了PPA的临床和神经心理学特征 ;并进行了MRI和PET影像学检查。结果　病人除有单纯性命名性失语外 ,不伴有其他类型的失语与智能损害及神经系统体征 ;MRI和PET检查均发现左颞叶明显萎缩。结论　PPA以缓慢进行性失语而不伴有认知功能障碍和神经系统体征为特点 ,优势半球局灶性额、颞叶病变有诊断意义。 Objective Primary progressive aphasia (PPA) is a rare central nervous system degenerative disease, which is rarely reported in China. One case is reported to improve the clinician’s understanding of the disease. Methods A comprehensive set of neuropsychological measures such as Wechsler Memory Scale, Cognitive Ability Screening Test, Building Block Test, Digital Breadth Test and Social Function Questionnaire were used to examine and describe the clinical and neuropsychological features of PPA. MRI And PET imaging examination. Results In addition to simple nominal aphasia, the patient was not associated with other types of aphasia and intellectual impairment and neurological signs. MRI and PET findings showed a significant atrophy of the left temporal lobe. Conclusion PPA is characterized by slow progressive aphasia without cognitive dysfunction and neurological signs. The dominant hemispheric frontal and temporal lobe lesions are of diagnostic significance.