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偶发性和家族性 (8型) 肌缩侧面硬化症中的语言障碍:一项比较研究

Caroline M de Araújo¹, Cássia de Alcântara¹, Mariana A Alencar²

¹Postgraduate Program in Neuroscience, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil.

|May 13, 2024

概括

患有家族型8型ALS (ALS8) 的患者表现出类似于零星ALS的语言缺陷,影响理解和言论. 这些非运动症状独立于运动功能.

科学领域:

神经科学是一个神经科学.
语言学的语言学.
遗传学遗传学是一个

背景情况:

偶发性肌缩侧面硬化症 (sALS) 经常影响语言功能,包括命名,理解和表达.
与p.P56S VAPB突变相关的8型家族性ALS (ALS8) 的特定语言概况尚未确定.

研究的目的:

调查和描述家族ALS8.8患者的语言缺陷.
将ALS8患者的语言表现与sALS患者和健康对照者的语言表现进行比较.

主要方法:

对三个组进行了全面的语言评估:sALS患者 (n=20),家族ALS8患者 (n=22) 和健康对照组 (n=21).
评估包括听觉理解,口头表达,流利性,语义记忆和话语分析的标准化测试.
参与者还完成了认知,焦虑和抑郁等级.

主要成果:

与对照组相比,SALS和ALS8患者表现出口语理解 (语法和语音处理) 和话语凝聚力受损.
在sALS和ALS8患者组之间没有观察到语言表现的显著差异.
语言缺陷与功能性或精神病学评估得分没有相关性.

结论:

家庭ALS8患者表现出与运动神经元退化不同的语言障碍.
这些发现表明,ALS8的特点是显著的非运动特征,特别是在语言处理.
在ALS8中,语言缺陷与疾病严重程度或运动障碍无关.

关键词:

这就是VAPB.骨髓缩侧面硬化症 (ALS) 是一种认知认知认知语言障碍语言障碍

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