原发性进行性失语1例报道

Clinical report of a case of primary progressive aphasia

目的报道1例原发性进行性失语(PPA)患者的言语障碍及影像学特点,探讨PPA的临床特征。方法采用汉语失语成套测验(ABC)中的口语流利性评定标准评估患者的口语流利性,采用西部失语成套测验评定患者的失语症类型,采用波士顿诊断性失语严重程度分级标准对患者失语症的严重程度进行分级,采用视觉词-图匹配、图形写名及口语阅读等认知心理学测试判定患者是否存在动-名词分离,同时进行记忆力筛查及影像学检查。结果患者自发谈话为流利型,有语义错语;失语症类型为感觉性失语,失语严重程度为4级。患者视觉词-图匹配和口语阅读测验显示,动词和名词的正确率比较,差异无统计学意义(P>0.05);而图形写名测验显示,动、名词书写正确率比较差异有统计学意义(P<0.05)。患者无记忆力障碍,MRI显示左侧额叶及颞叶萎缩,功能磁共振显示左侧额叶、颞叶呈低灌注、低代谢状态。结论本例PPA患者的临床表现以言语障碍为突出,出现动词特异性损伤,病变部位主要位于左侧颞叶与额叶,且该两部位呈低灌注、低代谢状态。

Objective To report the language disorder and imaging characteristics of a patient with primary progressive aphasia (PPA), and to investigate the clinical features of PPA. Methods The oral fluency subscale of Aphasia Battery of China (ABC), the Western Aphasia Battery (WAB), grade criterion of the Boston Diagnostic Aphasia (BDA) and cognitive psychology tests (CPT), including visual words/pictures matching (VPM) and nouns, verbs/pictures naming (VPN) and reading tasks (RT), were used to assess the fluency of oral language, the type and degree of aphasia, and verb-noun separation. Meanwhile, the faculty of memory was measured and analyzed by use of imaging scan. Results The patient spoke fluently and had some semantic paraphasia, the type was sensory aphasia, and the grade was four. There was no significant deviation between verb and noun correct rate in VPM and RT (P>0.05), while there was significant difference between verb and noun correct rate in VPN (P<0.05). The patient′s memory and intelligence were normal. Atrophy of left temporal and frontal lobes was shown by MRI, and the lower metabolism and blood flow of them were revealed by use of functional magnetic imaging. Conclusion Language disorder is a predominant characteristics of this PPA patient, who had verb specific deficits. And the major lesion regions were left temporal and frontal lobes with lower metabolism and blood flow.