Abstract

Background: Language dysfunction is a feature of cognitive impairment in amyotrophic lateral sclerosis (ALS) that may compromise communication. Objective: To elucidate language dysfunction in patients with ALS and its relationship with other neuropsychological tests and to identify the brain regions associated with this dysfunction using perfusion image. Methods: Overall, 37 patients with ALS were included in this study. Their neuropsychological function was investigated using the Western Aphasia Battery (WAB), Frontal Assessment Battery and Behavioral Assessment of the Dysexecutive Syndrome. N-isopropyl-p-[¹²³I] iodoamphetamine single-photon emission computed tomography was used to examine regional cerebral blood flow and its relationship with WAB scores was investigated using multiple regression analyses, controlled for age, sex and years of education. Results: Frequency of language abnormality in ALS was 8.5% for spontaneous speech, 25.7% for auditory verbal comprehension, 8.8% for repetition, 14.7% for naming, 17.6% for reading and 51.4% for writing. The writing error was mainly omission and substitution of kana letters. Executive tests were correlated with naming (r > 0.5, p < 0.001) and reading (r > 0.4, p < 0.01) scores. With respect to the writing sub-test, positive perfusional relationship was only detected in the left angular gyrus. Conclusions: The left angular gyrus is the region associated with the writing errors observed in ALS.

摘要

背景：语言障碍是肌萎缩性侧索硬化症（ALS）认知障碍的一个特征，可能会损害沟通。目的：阐明ALS患者的语言功能障碍及其与其他神经心理学测试的关系，并使用灌注图像识别与该功能障碍相关的大脑区域。方法：本研究共纳入37例ALS患者。他们的神经心理功能进行了调查，使用西方失语症电池（WAB），额叶评估电池和行为障碍综合症的行为评估。N-异丙基-对-[ ¹²³I]碘苯丙胺单光子发射计算机断层扫描用于检查区域性脑血流量，并使用年龄，性别和受教育年限进行的多元回归分析，研究了其与WAB评分的关系。结果： ALS的语言异常频率为自发性语音的8.5％，听觉言语理解的25.7％，重复的8.8％，命名的14.7％，阅读的17.6％和写作的51.4％。写作错误主要是假名字母的遗漏和替代。执行测试与命名（r  > 0.5，p  <0.001）和阅读（r  > 0.4，p <0.01）分。关于写作子测验，仅在左角回中检测到正灌注关系。结论：左角回是与ALS中观察到的书写错误相关的区域。