Yamamoto Toshiyuki, Kikuchi Takeshi, Nagae Junko, Ogata Katsuhisa, Ogawa Masafumi, Kawai Mitsuru
Rinsho Shinkeigaku. 2004 Jan;44(1):28-33.
A 60-year-old right-handed man showed dysprosody and agnosia for environmental sounds. His mother tongue was Japanese, and he could not speak foreign languages. He gradually developed difficulty in speaking from the age of 57 years, speaking non-native Japanese. In addition, he often complained of difficulty in hearing sounds, but audiometry showed no abnormalities. At the age of 60 years, the standard language test of aphasia showed no abnormalities in repetition, verbal comprehension, or reading, suggesting the absence of aphasia. However, in speaking, marked abnormality in rhythm, and occasional lack of postpositional particles and syllable-stumblings were observed. Writing was almost accurate, but a few grammatical errors were observed in speaking were observed. There were no cerebellar symptoms, pyramidal signs, pathologic reflexes, or abnormalities in phonation-related organs. Though the recognition of verbal sounds was maintained, impairment in the recognition of non-verbal sounds was observed. An environmental sound perception test showed correct answers only in 8 of 21 non-verbal sound sources (such as a car starting, glass breaking and so on), suggesting agnosia for environmental sounds. He insisted that the difficulty in perception was due to hearing impairment. However, re-examination with an increase in the sound volume showed similar results. He had no inconvenience in daily life and was not aware of agnosia for environmental sounds. He could recognize and differentiate sounds he heard once. His intelligence was normal, and neither apraxia nor frontal lobe symptoms were observed. MRI of the brain revealed slight atrophy of the right temporal lobe. Cerebral blood flow SPECT showed decreased blood flow from the superior temporal gyrus to the area around the arcuate fasciculi in the right temporal lobe. We considered that the lesion responsible for environmental auditory sound agnosia was present in the area around the secondary auditory area of the right temporal lobe and this patient differed from slowly progressive aphasia characterized by decreased blood flow in the left temporal lobe. Although the pathological process occurring in the area of hypoperfusion remained unclear, early stage of some degenerative disorders was more likely than cerebrovascular disease.
一名60岁的右利手男性表现出韵律障碍和对环境声音的失认。他的母语是日语,不会说外语。从57岁开始,他逐渐出现说非母语日语的困难。此外,他经常抱怨听力困难,但听力测试未显示异常。60岁时,失语症的标准语言测试显示重复、言语理解或阅读方面无异常,提示无失语症。然而,在说话时,观察到明显的节奏异常,偶尔缺少后置词和音节卡顿。书写基本准确,但说话时观察到一些语法错误。没有小脑症状、锥体束征、病理反射或发声相关器官的异常。虽然言语声音的识别得以保留,但观察到非言语声音识别受损。一项环境声音感知测试显示,在21种非言语声音来源(如汽车启动、玻璃破碎等)中,只有8种能正确回答,提示对环境声音失认。他坚称感知困难是由于听力障碍。然而,增加音量后的复查显示结果相似。他日常生活中没有不便,也没有意识到对环境声音的失认。他能识别和区分曾经听到过的声音。他智力正常,未观察到失用症或额叶症状。脑部MRI显示右侧颞叶轻度萎缩。脑血流SPECT显示右侧颞叶从颞上回至弓状束周围区域的血流减少。我们认为,导致环境听觉失认的病变位于右侧颞叶次级听觉区周围,且该患者与以左侧颞叶血流减少为特征的缓慢进行性失语症不同。虽然灌注不足区域发生的病理过程尚不清楚,但某些退行性疾病的早期阶段比脑血管疾病更有可能。