亨廷顿病的冷漠:当前概念的回顾

M. Camacho, R. Barker, S. Mason
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引用次数: 13

摘要

冷漠是亨廷顿舞蹈病(HD)患者最常见的精神症状之一。它出现得早,随着病程的发展而发展,并已被证明对照顾者的负担有重大影响。然而,对HD中冷漠的理解并不明确,其背后的机制也不清楚。在这篇综述中,我们讨论了HD背景下的冷漠概念,并提出关于其概念化和随后的诊断标准的共识将大大有利于该领域。为了开展这项工作,我们首先回顾了HD中冷漠的定义和评估的现有文献,其潜在的神经生物学基础及其与其他相关特征(如缺语、快感缺乏和述情障碍)的关系。在HD的背景下,冷漠可以被描述为动机、情感和目标导向行为的丧失或减少,而这并不能最好地用疾病的运动或社会限制来解释。然而,迫切需要更好地了解HD中冷漠的特征,它们如何在整个疾病过程中进化,以及它们如何与中枢多巴胺能通路相关。只有开展这样的工作,我们才能希望更好地了解HD的早期和致残方面。
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Apathy in Huntington’s Disease: A Review of the Current Conceptualization
Apathy is one of the most common psychiatric symptoms experienced by patients with Huntington’s disease (HD). It appears early, progresses with the disease course and has been shown to contribute significantly to caregiver burden. However, what is understood by apathy in HD is not clearly defined nor the underlying mechanisms responsible for it. In this review, we discuss the concept of apathy in the context of HD and propose that a consensus regarding its conceptualisation and subsequently its diagnostic criteria would significantly benefit the field. In order to undertake this work, we began by reviewing the existing literature on the definition and assessment of apathy in HD, its underlying neurobiological basis and its relationship to other related features such as abulia, anhedonia and alexithymia. In the context of HD, apathy could be described by a loss of or diminished motivation, emotion and goal-directed behaviour that is not best explained by motor or social constraints of the disease. However, there is an urgent need to better understand the characteristics of apathy specifically in HD, how they evolve across the disease course and how they relate to central dopaminergic pathways. Only by undertaking such work can we hope to better understand this early and disabling aspect of HD.
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