Katz D I, Alexander M P, Mandell A M
Department of Neurology, Boston University School of Medicine, MA.
Arch Neurol. 1987 Nov;44(11):1127-33. doi: 10.1001/archneur.1987.00520230017007.
Six patients had ischemic infarcts in the paramedian thalamic, subthalamic, and mesencephalic areas. In addition to ocular motility problems, ataxia, dysmetria, and mild pyramidal signs, there were consistent behavioral observations and neuropsychological findings. All of the patients had initial deficits in arousal, and gradually improved to normal wakefulness. When awake, all of the patients had impaired attention, mental control, and slowed verbal and motor responsiveness. They were apathetic, poorly motivated, and affect was flat or occasionally labile. All of the patients had a memory disorder characterized by anterograde and retrograde loss. One patient had significant language impairment. These deficits persisted in all but one patient who had a predominantly mesencephalic lesion. We believe the cluster of findings in these patients constitutes a characteristic syndrome of dementia related to paramedian mesencephalic and diencephalic infarcts. This syndrome bears close resemblance to that associated with some subcortical degenerative disorders such as progressive supranuclear palsy. In cases of paramedian mesencephalic and diencephalic infarcts, however, computed tomography and magnetic resonance imaging can delineate clinicoanatomic relationships that account for specific constituents of the syndrome.
6例患者在丘脑旁正中、丘脑底和中脑区域出现缺血性梗死。除眼球运动问题、共济失调、辨距不良和轻度锥体束征外,还有一致的行为观察结果和神经心理学发现。所有患者最初均有觉醒障碍,并逐渐改善至正常清醒状态。清醒时,所有患者均存在注意力、精神控制受损,言语和运动反应迟缓。他们表现冷漠,动力不足,情感平淡或偶尔不稳定。所有患者均有以顺行性和逆行性遗忘为特征的记忆障碍。1例患者有明显的语言障碍。除1例主要为中脑病变的患者外,这些缺陷在所有患者中均持续存在。我们认为这些患者的一系列发现构成了一种与丘脑旁正中及间脑梗死相关的痴呆特征性综合征。该综合征与某些皮质下变性疾病如进行性核上性麻痹相关的综合征极为相似。然而,在丘脑旁正中及间脑梗死的病例中,计算机断层扫描和磁共振成像能够描绘出解释该综合征特定组成部分的临床解剖关系。