Roig M, Calopa M, Rovira A, Macaya A, Riudor E, Losada M
Child Neurology Unit, Vall D'Hebron University Hospital, Autonomous University of Barcelona, Spain.
Pediatr Neurol. 1993 Sep-Oct;9(5):349-58. doi: 10.1016/0887-8994(93)90103-j.
From 1983 to 1991, 13 patients were identified with a clinical radiologic association characterized by acute or persistent neurologic dysfunction and bilateral lesions in the basal ganglia region demonstrated by ultrasound, computed tomography, or magnetic resonance imaging. Initial clinical manifestations of this group of patients were characterized by extrapyramidal signs (i.e., dystonia 9, hypotonia 2, athetosis 1, rigidity 1), altered state of consciousness in 5, and seizures in 3. The outcomes of most of these patients were poor: 10 had motor sequelae, 9 cognitive impairment, and 4 died. The outcomes of 2 patients, however, were much better than what was expected from the initial presentation. Based on current and previous reports, the diagnostic approach and classification of patients with neurologic dysfunction and bilateral striatal lesions are presented.
1983年至1991年期间，共确定了13例患者，其临床放射学关联特征为急性或持续性神经功能障碍，超声、计算机断层扫描或磁共振成像显示基底节区有双侧病变。该组患者的初始临床表现以锥体外系体征（即肌张力障碍9例、肌张力减退2例、手足徐动症1例、强直1例）、意识状态改变5例和癫痫发作3例为特征。这些患者中的大多数预后较差：10例有运动后遗症，9例有认知障碍，4例死亡。然而，2例患者的预后比最初表现预期的要好得多。基于当前和既往报告，本文介绍了神经功能障碍和双侧纹状体病变患者的诊断方法和分类。
