在原发性侧面硬化和遗传性性残中,上部运动神经元的征兆
Cláudia Santos Silva1,2, Catarina Correia Rodrigues1, Maria Fortuna Baptista1
1Department of Neurosciences and Mental Health, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa-Norte, Lisbon, Portugal.
Muscle & nerve
|April 30, 2024
概括
上部运动神经元 (UMN) 症状在初级侧面硬化症 (PLS) 中很常见,特别是在上肢. 不对称的UMN症状和上肢可有助于区分PLS和遗传性性 (HSP).
科学领域:
- 神经学 神经学
- 神经科学是一个神经科学.
- 临床医学 临床医学
背景情况:
- 初级侧面硬化 (PLS) 是一种罕见的,成人发病的运动神经元疾病,其特征是渐进的上部运动神经元 (UMN) 功能障碍.
- 在PLS中,UMN标志的精确频率和分布模式尚未确立,这阻碍了差异诊断.
- 遗传性性 (HSP) 与PLS有一些临床特征,需要明确的诊断标记.
研究的目的:
- 确定PLS患者UMN症状的频率和频谱.
- 调查UMN信号分布与临床特征之间的关系,例如PLS的发病区域和症状持续时间.
- 将PLS的UMN标志配置文件与HSP的UMN标志配置文件进行比较,以确定区分特征.
主要方法:
- 在34名PLS患者和20名HSP患者中,对UMN症状 (过度反射,性,病理反射) 的回顾性分析.
- 在第一次临床观察时评估UMN症状,将发现与发病区域和疾病持续时间相关联.
- 基于发病和持续时间的PLS和HSP队列之间的比较统计分析,以及PLS子组内的比较统计分析.
主要成果:
- 上肢 (UL) 高反射 (88.2%) 和下肢 (LL) 高反射 (91.2%) 在PLS中最常见. 此外,LL性 (79.4%) 也很常见.
- 根据发病区域,UMN标志的分布有所不同:在LL-脊柱发病时的LL性,在柱发病时的舌头性,以及在UL-脊柱发病次组中的霍夫曼标志.
- 与HSP患者相比,PLS患者表现出明显更多的UL超反射和UL性. 在PLS中观察到不对称的UMN标志,但在HSP中没有.
结论:
- 在初始呈现时,UMN标志,特别是在上肢,在PLS中几乎普遍存在.
- 在PLS中UMN标志的分布与症状发作的区域有关.
- 凸轮干涉,不对称的UMN标志,以及在第一次观察时的UL是区分PLS和HSP的潜在指标.
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