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日本肌萎缩侧索硬化症患者因额叶功能障碍导致的书写错误。

Writing errors as a result of frontal dysfunction in Japanese patients with amyotrophic lateral sclerosis.

机构信息

Department of Neurology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

出版信息

J Neurol. 2010 Dec;257(12):2071-7. doi: 10.1007/s00415-010-5662-9. Epub 2010 Jul 25.

Abstract

Loss of communication is a critical problem for advanced amyotrophic lateral sclerosis (ALS) patients. This loss of communication is mainly caused by severe dysarthria and disability of the dominant hand. However, reports show that about 50% of ALS patients have mild cognitive dysfunction, and there are a considerable number of case reports on Japanese ALS patients with agraphia. To clarify writing disabilities in non-demented ALS patients, eighteen non-demented ALS patients and 16 controls without neurological disorders were examined for frontal cognitive function and writing ability. To assess writing errors statistically, we scored them on their composition ability with the original writing error index (WEI). The ALS and control groups did not differ significantly with regard to age, years of education, or general cognitive level. Two patients could not write a letter because of disability of the dominant hand. The WEI and results of picture arrangement tests indicated significant impairment in the ALS patients. Auditory comprehension (Western Aphasia Battery; WAB IIC) and kanji dictation also showed mild impairment. Patients' writing errors consisted of both syntactic and letter-writing mistakes. Omission, substitution, displacement, and inappropriate placement of the phonic marks of kana were observed; these features have often been reported in Japanese patients with agraphia resulted from a frontal lobe lesion. The most frequent type of error was an omission of kana, the next most common was a missing subject. Writing errors might be a specific deficit for some non-demented ALS patients.

摘要

交流障碍是晚期肌萎缩侧索硬化症(ALS)患者面临的一个关键问题。这种交流障碍主要是由严重的构音障碍和优势手残疾引起的。然而,有报道称约 50%的 ALS 患者存在轻度认知功能障碍,并且有相当数量的关于日本 ALS 患者失写症的病例报告。为了阐明非痴呆 ALS 患者的书写障碍,我们对 18 名非痴呆 ALS 患者和 16 名无神经系统疾病的对照者进行了额叶认知功能和书写能力检查。为了对书写错误进行统计学评估,我们使用原始书写错误指数(WEI)对其作文能力进行评分。ALS 组和对照组在年龄、受教育年限或一般认知水平方面无显著差异。有两名患者因优势手残疾而无法书写字母。WEI 和图片排列测试结果表明 ALS 患者存在明显的障碍。听觉理解(西方失语症成套测验;WAB IIC)和汉字听写也表现出轻度损伤。患者的书写错误包括语法和字母书写错误。省略、替代、移位和 kana 语音标记的不当放置都有观察到;这些特征在由额叶病变引起的日本失写症患者中经常报道。最常见的错误类型是省略 kana,其次是缺少主语。书写错误可能是一些非痴呆 ALS 患者的特定缺陷。

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