Alexander Bampton, Caroline McHutchison, Kevin Talbot, Michael Benatar, Alexander G. Thompson, Martin R. Turner
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引用次数: 0
Abstract
Objective
To summarize and evaluate evidence pertaining to the clinical, genetic, histopathological, and neuroimaging correlates of cognitive and behavioral dysfunction in amyotrophic lateral sclerosis (ALS).
Methodology
We comprehensively reviewed the literature on cognitive and behavioral manifestations of ALS, narrating findings from both cross-sectional and longitudinal studies. We discussed knowledge gaps in the evidence base and key limitations affecting studies to date, before formulating a framework for future research paradigms aimed at investigating clinicopathological correlates of neuropsychological dysfunction in ALS.
Results
Studies have demonstrated clinical associations with cognitive dysfunction in ALS e.g., bulbar-onset of symptoms, pathological associations (extramotor TDP-43 deposition), and imaging associations (frontotemporal involvement). The most common behavioral deficit, apathy, is highly associated with verbal fluency, but longitudinal studies assessing behavioral dysfunction in ALS are comparatively lacking.
Conclusion
Longitudinal studies have been helpful in identifying several potential correlates of cognitive and behavioral dysfunction but have frequently been confounded by selection bias and inappropriate testing platforms. This review provides a framework for more robust assessment of clinicopathological associations of neuropsychological abnormalities in ALS in the future, advocating for greater utilization of pre-symptomatic C9orf72 repeat expansion-carrying cohorts.
目的总结并评估与肌萎缩侧索硬化症(ALS)认知和行为功能障碍的临床、遗传、组织病理学和神经影像学相关的证据:我们全面回顾了有关 ALS 认知和行为表现的文献,叙述了横断面和纵向研究的结果。我们讨论了证据基础中的知识空白和影响迄今为止研究的主要局限性,然后制定了未来研究范式的框架,旨在调查 ALS 神经心理功能障碍的临床病理相关性:研究表明,肌萎缩性脊髓侧索硬化症的认知功能障碍与临床相关,如横杆症状、病理学相关(运动外TDP-43沉积)和影像学相关(额颞叶受累)。最常见的行为缺陷--冷漠与语言流畅性高度相关,但评估 ALS 行为功能障碍的纵向研究相对缺乏:纵向研究有助于确定认知和行为功能障碍的几个潜在相关因素,但经常受到选择偏差和不适当的测试平台的影响。本综述为今后更有力地评估 ALS 神经心理异常的临床病理关联提供了一个框架,提倡更多地利用无症状前 C9orf72 重复扩增携带队列。
期刊介绍:
Brain and Behavior is supported by other journals published by Wiley, including a number of society-owned journals. The journals listed below support Brain and Behavior and participate in the Manuscript Transfer Program by referring articles of suitable quality and offering authors the option to have their paper, with any peer review reports, automatically transferred to Brain and Behavior.
* [Acta Psychiatrica Scandinavica](https://publons.com/journal/1366/acta-psychiatrica-scandinavica)
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