肌缩侧面硬化症认知功能障碍的频谱:一个更新
1Institute of Clinical Neurobiology, Alberichgasse 5/13, A-1150 Vienna, Austria.
International journal of molecular sciences
|October 14, 2023
概括
肌缩侧面硬化 (ALS) 的认知功能障碍影响30-75%的患者,从轻度下降到前性痴呆 (FTD). 早期发现大脑连接变化可能会提供新的治疗点.
科学领域:
- 神经科学是一个神经科学.
- 神经学 神经学
- 认知科学 认知科学
背景情况:
- 认知功能障碍是肌缩侧面硬化症 (ALS) 的一个显著的非运动症状.
- 它对患者的生存率和护理人员的负担产生负面影响,呈现从主观衰退到前性痴呆 (FTD) 的范围.
- 在ALS的认知缺陷主要涉及执行功能,注意力,语言和口头记忆.
研究的目的:
- 探索ALS认知功能障碍的频谱和潜在机制.
- 研究认知障碍,神经成像发现和ALS遗传/病理异质性之间的关系.
- 确定潜在的早期生物标志物和ALS认知障碍的治疗标.
主要方法:
- 对现代神经成像研究 (例如fMRI,PET) 的审查,检查ALS患者的大脑结构和功能.
- 对遗传,临床和病理数据的分析,包括TDP-43和tau病理.
- 认知储备的评估及其在缓解认知衰退中的作用.
主要成果:
- 神经成像显示了前退行,边缘和白质系统的参与,以及相关大脑区域的低代谢.
- 形态变化包括带有突触损失的前腔和海马缩,与TDP-43和病理相关.
- 运动/超运动网络的功能性障碍和改变的前/前连接与认知缺陷相关.
结论:
- 在ALS中认知障碍是异质的,与特定的神经退行性模式和病理有关.
- 虽然认知储备可能起到作用,但它不能完全防止衰退.
- 早期发现大脑连接变化可以阐明病理生理机制,并指导ALS相关认知功能障碍的新疗法策略.
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