Action selection is a capital feature of cognition that guides behavior in processes that range from motor patterns to executive functions. Here, the ongoing actions need to be monitored and adjusted in response to sensory stimuli to increase the chances of reaching the goal. As higher hierarchical processes, these functions rely on complex neural circuits, and connective loops found within the brain and the spinal cord. Successful execution of motor behaviors depends, first, on proper selection of actions, and second, on implementation of motor commands. Thus, pathological conditions crucially affecting the integrity and preservation of these circuits and their connectivity will heavily impact goal-oriented motor behaviors. Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD) are two neurodegenerative disorders known to share disease etiology and pathophysiology. New evidence in the field of ALS-FTD has shown degeneration of specific neural circuits and alterations in synaptic connectivity, contributing to neuronal degeneration, which leads to the impairment of motor commands and executive functions. This evidence is based on studies performed on animal models of disease, post-mortem tissue, and patient derived stem cells. In the present work, we review the existing evidence supporting pathological loss of connectivity and selective impairment of neural circuits in ALS and FTD, two diseases which share strong genetic causes and impairment in motor and executive functions.

中文翻译：

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ALS-FTD 病理学中的神经回路和突触功能障碍

行动选择是认知的一个重要特征，它指导从运动模式到执行功能等过程中的行为。在这里，需要监控和调整正在进行的行动以响应感官刺激，以增加实现目标的机会。作为更高层次的过程，这些功能依赖于复杂的神经回路以及大脑和脊髓内的连接环。运动行为的成功执行首先取决于动作的正确选择，其次取决于运动命令的执行。因此，病理状况对这些回路及其连接的完整性和保存至关重要，将严重影响目标导向的运动行为。肌萎缩侧索硬化症 (ALS) 和额颞叶痴呆 (FTD) 是两种已知具有相同病因和病理生理学的神经退行性疾病。ALS-FTD 领域的新证据表明，特定神经回路的退化和突触连接的改变，导致神经元退化，从而导致运动命令和执行功能受损。该证据基于对疾病动物模型、死后组织和患者来源的干细胞进行的研究。在目前的工作中，我们回顾了支持 ALS 和 FTD 病理性连接性丧失和神经回路选择性损伤的现有证据，这两种疾病具有强烈的遗传原因以及运动和执行功能损伤。