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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。
Background: Primary progressive aphasia is a nervous system degenerative disease, which is rare in clinic. Only 3 cases were reported in China. On the primary progressive aphasia of speech and imaging features, less clinical data. Objective: To report the speech impairment and imaging features of a patient with primary progressive aphasia and to describe the clinical features of primary progressive aphasia. Design: Case Analysis. SETTING: Department of Neurology, Beijing Tiantan Hospital, School of Psychology, Beijing Normal University. Subjects: male patient, 56 years old, graduated from high school, before the onset of business. The main reason for “verbal ability decreased 3 years” in Beijing Tiantan Hospital, neurology, diagnosis of primary progressive aphasia. Methods: The patients were evaluated fluently in spoken Chinese in Aphasia at the First Hospital of Beijing Medical University. The severity of aphasia was graded using a western aphasia test to determine the type of aphasia and the diagnostic criteria of the severity of aphasia in Boston. Memory, intelligence screening and imaging studies. MAIN OUTCOME MEASURES: The patient’s oral fluency, the type of aphasia, the severity of aphasia, whether memory and mental retardation, imaging features. Results: The patient was fluent spoken English. The western aphasia test was assessed as sensory aphasia. The severity of aphasia was grade 4 with no memory loss and mental retardation. MRI showed left frontal lobe and temporal lobe atrophy with obvious left and functional magnetic Resonance showed that the left temporal lobe and the frontal lobe showed a low metabolism and hypoperfusion state. Conclusion: Aphasic progressive aphasia is characterized by verbal dysfunction with prominent clinical manifestations. There is no other cognitive dysfunction at the onset of the disease. The lesion mainly lies in the left temporal lobe and the frontal lobe.