Iroi Atsuko, Okuma Yasuyuki, Fukae Jiro, Fujishima Kenji, Goto Keigo, Mizuno Yoshikuni
Department of Neurology, Juntendo Izu-Nagaoka Hospital, 1129 Nagaoka, Izu-Nagaoka, Tagata-gun, Shizuoka 410-2295, Japan.
No To Shinkei. 2002 Oct;54(10):903-7.
We report a 63-year-old right-handed Japanese man with progressive bulbar dysfunction and alexia of kanji (Japanese morphograms). He was well until his 62 years of age, when he noted difficulty of reading kanji, which was followed by disturbances in his speech. Reading of kana (Japanese phonograms) was preserved. He also showed naming difficulties with semantic memory loss for words, which were characterized for word meaning aphasia or semantic dementia. He showed dysarthria and mild dysphagia with atrophy and fasciculations of the tongue. The electromyographic studies disclosed diffuse neurogenic pattern. He was diagnosed as having bulbar type amyotrophic lateral sclerosis. Cranial magnetic resonance imaging and single-photon emission computed tomography revealed bilateral involvements of the temporal lobes. Our patient appeared to meet the clinical criteria for frontotemporal degeneration of motor neuron disease type, and is the first case of amyotrophic lateral sclerosis showing alexia of kanji and word meaning aphasia.
我们报告了一名63岁的右利手日本男性，患有进行性延髓功能障碍和汉字失读症（日语形态图）。他在62岁之前身体状况良好，当时他注意到阅读汉字有困难，随后出现了言语障碍。假名（日语音标）的阅读能力得以保留。他还表现出命名困难以及对单词的语义记忆丧失，其特征为词义性失语或语义性痴呆。他出现了构音障碍和轻度吞咽困难，伴有舌肌萎缩和肌束震颤。肌电图研究显示为弥漫性神经源性模式。他被诊断为延髓型肌萎缩侧索硬化症。头颅磁共振成像和单光子发射计算机断层扫描显示双侧颞叶受累。我们的患者似乎符合运动神经元病型额颞叶变性的临床标准，并且是首例表现出汉字失读症和词义性失语的肌萎缩侧索硬化症病例。
