调查年轻和晚期发作的渐进性超核性麻之间的差异
Batoul A AlWazan1,2, Indira Garcia-Cordero3, Blas Couto4
1Memory Clinic, Toronto Western Hospital, Toronto, ON, Canada. Batoul.Alwazan@medportal.ca.
Journal of neurology
|September 5, 2023
概括
年轻发病的渐进性上核性麻 (PSP) 患者更有可能表现出言语和语言缺陷. 早期发病年龄可以预测特定的PSP表型,提供临床和预后见解.
科学领域:
- 神经学 神经学
- 神经退行性疾病 神经退行性疾病
- 临床的表型化 临床的表型化
背景情况:
- 发病年龄对渐进性上核性麻 (PSP) 现象型的影响仍未得到充分研究.
- 一个假设表明,年轻发病PSP (YOPSP) 和晚发病PSP (LOPSP) 之间存在不同的表现.
研究的目的:
- 为了比较YOPSP和LOPSP之间的临床表型和残疾进展率.
- 识别表现症状和满足诊断标准的差异.
主要方法:
- 根据MDS 2017标准对107名被诊断患有PSP的患者进行了回顾性分析.
- 根据65岁的年龄截止值将患者分为YOPSP和LOPSP.
- 对比了表型,症状,MDS核心标准和疾病严重程度 (PSP-RS).
主要成果:
- 在YOPSP患者中,PSP语音/语言 (SL) 表型 (18%vs0%) 和失言症 (16%vs1.4%) 的患病率更高.
- 语音和语言功能障碍 (C1) 在YOPSP中更常见 (33.3%对12.2%).
- 纵向PSP-RS数据表明,在6个月后,YOPSP中的圆柱体得分恶化更快.
结论:
- 年轻发病的PSP经常与语音和语言变异有关.
- 发病年龄是PSP表型的潜在预测因素,具有重要的临床和预后影响.
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