BACKGROUND: Primary progressive aphasia(PPA) is a degenerative disease of nervous system, which is very rare in clinics. Only 3 cases have been reported in our country. There is very little clinical information regarding the characteristics of PPA in linguistics and imageology.OBJECTIVE: To report the language disorder and the characteristics of imageology of one PPA patient for the clarification of the clinical features of PPA.DESIGN: A case analysis.SETTING: Department of Neurology, Beijing Tiantan Hospital; Faculty of Psychology, Beijing Normal University.PATICIPANT: Male, 56 years old, senior high school graduate, businessman before the onset of the disease. The case visited the department of Neurology,Tiantan Hospital, Beijing due to the complaint of three years of progressive decreasing in language skills, which was then diagnosed with PPA.METH ODS: Spoken language fluency evaluation in Chinese Aphasia Examination Set of the First Affiliated Hospital of Beijing Medical University was used to evaluate this patient, the type of Aphasia was judged by Western Aphasia Examination Set, and the severity gradation was classified by the Boston Diagnostic Aphasia Examination(BDAE) . Memory, intelligence screening and imageology examination were performed as well.MAIN OUTCOME MEASURES: The fluency of spoken language, the type of aphasia, and the severity of aphasia of the patient were judged, and whether the patient suffered from memory and intelligence disorder were observed as well as the features of imageology.RESULTS: The patient had fluent spoken language, which was evaluated as sensory aphasia(SA) with the aphasia severity of level 4, and had no memory or intelligence disorder. MRI showed atrophy in left frontal lobe and temporal lobe, which was more significant in left side. MRI also showed that left temporal and frontal lobes had low metabolism and low perfusion.CONCLUSION: PPA is a disease with language disorder as its dominant clinical manifestation, which no other cognitive disorder at its initial stage,and with its main pathological changes in left temporal and frontal lobes.%背景:原发性进行性失语是一种神经系统变性疾病,临床少见,国内仅报道了3例.关于原发性进行性失语的言语学及影像学特点,临床资料较少.目的:报道1例原发性进行性失语患者言语障碍及影像学特点,阐述原发性进行性失语的临床特征.设计:病例分析.单位:北京天坛医院神经内科、北京师范大学心理学院.对象:患者男,56岁,高中毕业,发病前经商.主因"言语能力进行性下降3年"就诊于北京天坛医院神经内科,诊断为原发性进行性失语.方法:对该例患者进行北京医科大学第一医院的汉语失语成套测验中口语流利性评定、应用西部失语成套测验判断失语症类型及波士顿诊断性失语严重程度分级标准进行失语症严重程度分级,进行记忆力、智力筛查及影像学检查.主要观察指标:该例患者的口语流利型,失语症类型,失语症的严重程度是否有记忆力及智力障碍,影像学特点.结果:患者为流利型口语,西部失语症成套测验评定为感觉性失语,失语严重程度为4级,无记忆力及智力障碍;MRI显示左侧额叶及颞叶萎缩,以左侧明显,功能磁共振显示左侧颞叶和额叶呈低代谢、低灌注状态.结论:原发性进行性失语以言语障碍为突出临床表现,发病初始无其他认知功能障碍,病变部位主要在左侧颞叶、额叶.
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