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On the pathophysiology and treatment of akinetic mutism.
Neuroscience & Biobehavioral Reviews ( IF 8.2 ) Pub Date : 2020-02-07 , DOI: 10.1016/j.neubiorev.2020.02.006
Hisse Arnts 1 , Willemijn S van Erp 2 , Jan C M Lavrijsen 3 , Simon van Gaal 4 , Henk J Groenewegen 5 , Pepijn van den Munckhof 1
Affiliation  

Akinetic mutism (AM) is a rare neurological disorder characterized by the presence of an intact level of consciousness and sensorimotor capacity, but with a simultaneous decrease in goal-directed behavior and emotions. Patients are in a wakeful state of profound apathy, seemingly indifferent to pain, thirst, or hunger. It represents the far end within the spectrum of disorders of diminished motivation. In recent years, more has become known about the functional roles of neurocircuits and neurotransmitters associated with human motivational behavior. More specific, there is an increasing body of behavioral evidence that links specific damage of functional frontal-subcortical organization to the occurrence of distinct neurological deficits. In this review, we combine evidence from lesion studies and neurophysiological evidence in animals, imaging studies in humans, and clinical investigations in patients with AM to form an integrative theory of its pathophysiology. Moreover, the specific pharmacological interventions that have been used to treat AM and their rationales are reviewed, providing a comprehensive overview for use in clinical practice.

中文翻译:

关于运动性默症的病理生理学和治疗。

运动性mut默症(AM)是一种罕见的神经系统疾病,其特征是存在完整的意识水平和感觉运动能力,但同时目标导向的行为和情绪却同时下降。患者处于清醒的冷漠状态,看似对疼痛,口渴或饥饿无动于衷。它代表了动机减弱的疾病范围内的远端。近年来,关于神经回路和神经递质与人类动机行为相关的功能性作用的了解越来越多。更具体地说,越来越多的行为学证据将功能性额叶-皮层下组织的特定损害与明显的神经功能缺损的发生联系起来。在这篇评论中,我们结合了病变研究的证据和动物的神经生理学证据,对人类进行影像学研究,并对AM患者进行临床研究,以形成其病理生理学的综合理论。此外,对已经用于治疗AM的特定药理干预措施及其原理进行了综述,为临床实践提供了全面的概述。
更新日期:2020-02-07
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