初级渐进性失语缺乏非流利和语义变体的核心特征:临床,神经成像和神经病理特征
Hiroyuki Watanabe1, Joseph R Duffy1, Heather Clark1
1From the Departments of Neurology (H.W., J.R.D., H.C., J.G.-R., K.A.J.), Psychology (M.M.M.), and Radiology (N.T.T.P., V.J.L., J.L.W.), Mayo Clinic, Rochester, MN; and Department of Neuroscience (Neuropathology) (D.W.D.), Mayo Clinic, Jacksonville, FL.
初级渐进性失言症 (PPA) 标准需要更新. 这项研究根据重复和理解重新分类了PPA亚型,揭示了与阿尔茨海默病相关的光谱,并提出了逻辑变异PPA (lvPPA) 的新标准.
科学领域:
- 神经学 神经学
- 神经科学是一个神经科学.
- 语音和语言病理学 语言病理学
背景情况:
- 对于原发性渐进性失言症 (PPA) 的现有2011年共识标准可能不完全涵盖逻辑变异PPA (lvPPA) 的临床谱.
- 需要完善诊断标准,以更好地使临床表现与潜在的神经病理学保持一致.
研究的目的:
- 检查缺乏非流动/语义变体特征的PPA的临床,神经成像和神经病理特征.
- 建议对2011年 PPA 的共识标准进行实际补充,特别是对于 lvPPA.
主要方法:
- 从两个观察性队列研究中对102名PPA患者进行了回顾性分析.
- 基于重复和理解缺陷的分类,分为纯LPA,韦尼克式,异常式和TCSA类子组.
- 神经成像 (MRI,18F-FDG-PET) 和神经病理学检查的一组患者.
主要成果:
- 重复保存的PPA (类似anomic,类似TCSA) 占队列的73%,而重复受损的PPA (纯LPA,类似Wernicke) 为27%.
- 分组代表了疾病过程的不同阶段,异常类型的PPA可能在其他形式之前出现.
- 阿尔茨海默氏病 (AD) 病理学普遍存在 (总体70%),特别是在重复受损的形式中,而异常类子组显示出更多样化的病理学.
结论:
- 没有非流动性/语义特征的PPA的临床异质性可以被视为AD相关的失语谱.
- 将中度/严重的重复缺陷纳入lvPPA标准可能会改善临床病理相关性.
- 识别渐进性异常失语症作为一个独特的PPA变体可以减少lvPPA的神经病理异质性.
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