临床尺寸沿着非流动变体的初级渐进性失语谱的临床尺寸
Ignacio Illán-Gala1,2,3, Diego L Lorca-Puls4,5, Boon Lead Tee4
1Sant Pau Memory Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona, 08025, Barcelona, Spain.
Brain : a journal of neurology
|November 21, 2023
概括
主要渐进式语音失语 (PPAOS) 和渐进式语法失语 (PAA) 似乎是临床连续的一部分,而不是不同的实体. 疾病的严重程度和异文主义预测了更快的进展,强调了更新分类的必要性.
科学领域:
- 神经科学是一个神经科学.
- 神经学 神经学
- 语音语言病理学 语言病理学
背景情况:
- 非流利/语法变异性初级渐进性失语症 (nfvPPA) 的分类仍在争论中,关于初级渐进性语音失语症 (PPAOS) 和渐进性语法失语症 (PAA) 是否是不同的或频谱的一部分的不确定性.
- 了解这些疾病的临床和病理相关性对于准确的诊断和预后至关重要.
研究的目的:
- 分析发言,语言和疾病严重程度在患有渐进性运动发言障碍和/或语法障碍的患者.
- 为了确定这种患者队列中是否存在PPAOS和PAA等不同的,不重叠的综合征实体.
- 识别潜在的病因或预后价值的数据驱动的潜在临床维度 (LCD).
主要方法:
- 对98名参与者的综合分析,包括43名尸检确诊的参与者,评估运动言语 (发作障碍,言语无力症 - AOS),语法障碍和疾病严重程度 (临床痴呆症评分总和盒 - CDR-SB).
- 利用集群和主要成分分析来识别症状集群和潜在的临床维度.
- 采用线性混合效应模型来分析纵向疾病进展.
主要成果:
- 只有两个强大的子组被确定,在语法学,执行功能障碍和疾病严重程度上有所不同,这表明临床连续性,而不是不同的PPAOS和PAA实体.
- 三个潜伏的临床维度解释了71%的差异:严重程度 - 异文主义,突出的AOS和突出的脱节性关节炎.
- 严重程度-阿格拉姆马蒂斯组件和基线脱关节/阿格拉姆马蒂斯预测疾病进展加速 (CDR-SB增速更快).
结论:
- PPAOS和PAA可能代表 nfvPPA频谱内的临床连续性.
- 目前的临床表型没有显著改善临床病理相关性,强调需要新的生物标志物和修订的分类系统.
- 疾病的进展是独立预测的因素,包括失联症的严重程度,言语速度,和语法水平.
关键词:
语音上的失言症 (apraxia of speech) 是一种语言失言症.皮层骨干退行症 皮层骨干退行症脱发性关节障碍症 (dysarthria) 是一种疾病.磁共振成像技术的使用主要的渐进性失言症.渐进性的超核性麻.更多相关视频
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