Acosta Lealani Mae Y, Goodman Ira J, Heilman Kenneth M
*Department of Neurology, Vanderbilt University, Nashville, TN †The Compass Clinic, Orlando, FL ‡Department of Neurology, University of Central Florida College of Medicine, Orlando, FL §Department of Neurology, University of Florida College of Medicine, Gainesville, FL ∥The Malcom Randall Veterans Affairs Medical Center, Gainesville, FL.
Cogn Behav Neurol. 2013 Dec;26(4):181-8. doi: 10.1097/WNN.0000000000000014.
The brain's action-intentional ("when") programming system helps to control when to and when not to initiate an action, when to persist at an action, and when to terminate an action. Motor perseveration is a failure to terminate an action. This disengagement disorder most often results from dysfunction of the executive frontal-subcortical networks that control the action-intentional programming system. Reports of unilateral perseveration are unusual. Here we describe a patient with a form of progressive supranuclear palsy (PSP) who exhibited continuous right-hand motor perseveration. This 68-year-old right-handed man had impaired walking and vertical gaze, consistent with PSP. He often repeated words, and on many motor tasks he showed continuous perseveration of his right but not his left hand. Unilateral motor perseveration may be a sign of PSP, the corticobasal syndrome, or a subtype of these disorders. Future studies of patients with both disorders should use tasks that assess for asymmetric hand perseveration. The mechanism of the unilateral perseveration must also be determined. Bilateral perseveration is found most often in patients with unilateral right frontal-subcortical (basal ganglia) or insula dysfunction. Because patients with PSP or corticobasal syndrome have callosal degeneration, their unilateral perseveration might result from a callosal disconnection of the right frontal lobe from the left hemisphere's premotor and motor as well as speech areas.
大脑的动作意图(“何时”)编程系统有助于控制何时启动动作、何时不启动动作、何时持续进行动作以及何时终止动作。动作持续是指无法终止动作。这种脱离障碍最常源于控制动作意图编程系统的额叶-皮质下执行网络功能障碍。单侧动作持续的报告并不常见。在此,我们描述了一名患有进行性核上性麻痹(PSP)的患者,其右手出现持续性动作。这位68岁的右利手男性存在行走和垂直凝视障碍,符合PSP的表现。他经常重复话语,并且在许多运动任务中,其右手而非左手表现出持续的动作持续。单侧动作持续可能是PSP、皮质基底节综合征或这些疾病的一种亚型的迹象。未来对患有这两种疾病的患者进行研究时,应使用评估手部不对称动作持续的任务。还必须确定单侧动作持续的机制。双侧动作持续最常见于单侧右额叶-皮质下(基底节)或岛叶功能障碍的患者。由于PSP或皮质基底节综合征患者存在胼胝体变性,他们的单侧动作持续可能是由于右额叶与左半球的运动前区、运动区以及言语区之间的胼胝体连接中断所致。