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The spectrum of language impairments in amyotrophic lateral sclerosis
Cortex ( IF 3.6 ) Pub Date : 2020-09-18 , DOI: 10.1016/j.cortex.2020.09.003
Amelia Ceslis 1 , Rosemary Argall 2 , Robert D Henderson 3 , Pamela A McCombe 3 , Gail A Robinson 4
Affiliation  

Language disorders are increasingly recognised in Amyotrophic lateral sclerosis (ALS), supporting the view of ALS as a multi-system disorder, impacting cognitive and motor function. However, the language impairments are heterogeneous and recent focus has been on determining the language profile across the ALS spectrum with little focus on spontaneous speech. The current study systematically investigated a wide range of language abilities in an unselected ALS sample (N = 22), including spontaneous speech. We analysed the ALS patients' performance as a group, compared to age-, education- and IQ-matched healthy controls (N = 21), and as a case series to identify dementia and specific language profiles. The ALS group was impaired on measures of spontaneous speech, word fluency and action naming. By contrast, object naming, semantic memory (object and actions), sentence comprehension and repetition (word and sentences) were comparable to healthy controls. In line with recent suggestions, our ALS patients’ action naming (but not action semantic) deficit does not support the notion that action processing may be selectively impaired in ALS. The case series demonstrated that 14% of patients had probable dementia, 31% showed significant cognitive and/or language impairment and 55% were unimpaired, consistent with the spectrum of cognitive and language impairments reported in the literature. In addition, 36% of ALS patients produced significantly fewer words per minute on a spontaneous speech task than the control group, with this difference remaining when the ALS patients with frontotemporal dementia were excluded from the analysis. This pattern was observed across the ALS spectrum and in both limb and bulbar onset patients. The pattern of performance observed in the present study suggests that spontaneous speech is reduced across the ALS spectrum even in those with intact core language abilities.



中文翻译:

肌萎缩性侧索硬化症的语言障碍谱

语言障碍在肌萎缩性侧索硬化症(ALS)中得到了越来越多的认可,这支持将ALS视为多系统障碍的观点,从而影响认知和运动功能。但是,语言障碍是多种多样的,最近的重点是确定整个ALS频谱的语言配置,而很少关注自发语音。当前的研究系统地研究了未经选择的ALS样本(N = 22)中的多种语言能力,包括自发言语。我们分析了与年龄,教育和智商相匹配的健康对照组(N = 21)相比,ALS患者作为一个整体的表现,并以此病例系列来识别痴呆和特定的语言特征。ALS小组在自发言语,单词流利程度和动作命名方面受到了损害。相比之下,对象命名,语义记忆(对象和动作),句子理解和重复(单词和句子)与健康对照相当。与最近的建议一致,我们的ALS患者的动作命名(但不是动作语义)缺陷并不支持在ALS中可能选择性损害动作处理的观点。该病例系列表明,14%的患者可能患有痴呆症,31%的患者表现出明显的认知和/或语言障碍,55%的患者未受损,这与文献中报道的认知和语言障碍的范围一致。此外,有36%的ALS患者在自发语音任务中每分钟产生的单词数明显少于对照组,当将额颞痴呆的ALS患者从分析中排除时,这种差异仍然存在。在整个ALS频谱以及四肢和延髓发作患者中都观察到了这种模式。在本研究中观察到的表现模式表明,即使在具有完整核心语言能力的人中,在ALS频谱上自发性语音也会减少。

更新日期:2020-10-05
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