Neuropathology of Auditory Agnosia Following Bilateral Temporal Lobe Lesions: A Case Study

Neuropathology of Auditory Agnosia Following Bilateral Temporal Lobe Lesions: A Case Study
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双侧颞叶病变后听觉失认症的神经病理学:案例研究

DOI:
10.1080/000164800750001053
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发表时间:
2000
影响因子:
1.4
通讯作者:
Kimitaka Kaga, Mitsuko Shindo, Yoshisato Tanaka, H
Kimitaka Kaga, Mitsuko Shindo, Yoshisato Tanaka, H
中科院分区:
医学4区
文献类型:
--
作者:
Kimitaka Kaga, Mitsuko Shindo, Yoshisato Tanaka, H

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我们的病人在1975年第二次脑血管意外(CVA)后首次被诊断为听觉失认症,当时他37岁。定期进行全面的听觉功能随访检查,直至15年后猝死。他的大脑在死后进行了神经病理学研究。最初的纯音测听显示右耳中度感音神经性听力损失,左耳轻度感音神经性听力损失。然而,重复的纯音测听显示,随着时间的推移,双侧阈值逐渐变差。双耳的言语测听始终显示患者无法辨别任何单音节单词(即,双侧言语清晰度评分为0%)。一般来说,语言和听力测试表明,他不能理解口语,但可以理解书面命令和手势。死后对左半球的神经病理学研究显示上级颞回(包括Heschl回)的完全缺损和神经元丢失,以及内侧膝状体的完全神经胶质增生。在右半球,检查发现皮质下坏死,上级颞回中心和内侧膝状体部分胶质增生。病理检查支持患者对语言声音、音乐和环境声音的感觉障碍可能是由于双侧内侧膝状体进行性变性所致的临床结果。
Our patient was first diagnosed with auditory agnosia following his second cerebral vascular accident (CVA) in 1975 when he was 37 years old. Comprehensive follow-up examinations of auditory function were periodically conducted until his sudden death 15 years later. His brain was studied postmortem for neuropathology. Initial pure-tone audiometry revealed moderate sensorineural hearing loss in the right ear and mild sensorineural hearing loss in the left ear. However, repeated pure-tone audiometry revealed that thresholds became progressively poorer over time, bilaterally. Speech audiometry of both ears consistently revealed that the patient was unable to discriminate any monosyllabic words (i.e. speech intelligibility scores were 0%, bilaterally). In general, speech and hearing tests demonstrated that he could not comprehend spoken words, but could comprehend written commands and gestures. Postmortem neuropathological study of the left hemisphere revealed total defect and neuronal loss of the superior temporal gyrus, including Heschl's gyrus, and total gliosis of the medial geniculate body. In the right hemisphere, examination revealed subcortical necrosis, gliosis in the centre of the superior temporal gyrus and partial gliosis of the medial geniculate body. The pathological examination supports clinical results in which the patient's imperception of speech sounds, music and environmental sounds could be caused by progressive degeneration of bilateral medial geniculate bodies.