Ehrlich Debra J, Walker Ruth H
Department of Neurology, Icahn School of Medicine at Mount Sinai, 5 East 98th Street, 1st Floor, Box 1637, New York, NY 10029 USA.
Department of Neurology, James J Peters Veterans Affairs Medical Center, 130 West Kingsbridge Road, Bronx, NY 10468 USA.
J Clin Mov Disord. 2017 Jun 21;4:8. doi: 10.1186/s40734-017-0056-0. eCollection 2017.
Chorea is a hyperkinetic movement disorder consisting of involuntary irregular, flowing movements of the trunk, neck or face. Although Huntington's disease is the most common cause of chorea in adults, chorea can also result from many other neurodegenerative, metabolic, and autoimmune conditions. While the pathophysiology of these different conditions is quite variable, recent advances in functional imaging have enabled the development of new methods for analysis of brain activity and neuronal dysfunction. In this paper we review the growing body of functional imaging data that has been performed in chorea syndromes and identify particular trends, which can be used to better understand the underlying network changes within the basal ganglia. While it can be challenging to identify whether changes are primary, secondary, or compensatory, identification of these trends can ultimately be useful in diagnostic testing and treatment in many of the conditions that cause chorea.
舞蹈症是一种运动亢进性疾病，表现为躯干、颈部或面部不自主的不规则、流畅运动。虽然亨廷顿舞蹈病是成人舞蹈症最常见的病因，但舞蹈症也可能由许多其他神经退行性、代谢性和自身免疫性疾病引起。尽管这些不同疾病的病理生理学差异很大，但功能成像的最新进展推动了分析脑活动和神经元功能障碍新方法的发展。在本文中，我们回顾了在舞蹈症综合征中进行的越来越多的功能成像数据，并确定了特定趋势，这些趋势可用于更好地理解基底神经节内潜在的网络变化。虽然识别这些变化是原发性、继发性还是代偿性具有挑战性，但识别这些趋势最终可能有助于许多导致舞蹈症的疾病的诊断测试和治疗。
