Ho V B, Fitz C R, Chuang S H, Geyer C A
Department of Radiology, Madigan Army Medical Center, Tacoma, WA 98431-5419.
Radiographics. 1993 Mar;13(2):269-92. doi: 10.1148/radiographics.13.2.8460220.
Computed tomography (CT) and magnetic resonance (MR) imaging have dramatically improved the ability to visualize the deep gray structures of the basal ganglia (primarily, the caudate nucleus, putamen, and globus pallidus). Any process that alters cerebral metabolism can lead to basal ganglia damage. This article presents the spectrum of disease that may be seen with bilateral basal ganglia abnormalities in the pediatric population. A simplified approach to the differential diagnosis of these entities is based on acute versus chronic conditions and radiologic manifestations. Acute processes include hypoxia, hypoglycemia, carbon monoxide poisoning, hemolytic-uremic syndrome, osmotic myelinolysis, and encephalitis. Chronic conditions include inherited ("inborn errors of metabolism," Huntington disease, and dysmyelinating diseases) or acquired (sequelae of acute disorders) conditions that represent abnormal biochemical or structural processes within the basal ganglia. Elimination of acute causes gives little hope for improvement. Recognition of chronic disorders is important for counseling purposes, since most of these conditions have specific patterns of inheritance.
计算机断层扫描（CT）和磁共振成像（MR）显著提高了可视化基底神经节深部灰色结构（主要是尾状核、壳核和苍白球）的能力。任何改变脑代谢的过程都可能导致基底神经节损伤。本文介绍了在儿科人群中双侧基底神经节异常可能出现的疾病谱。对这些实体进行鉴别诊断的一种简化方法基于急性与慢性情况以及放射学表现。急性过程包括缺氧、低血糖、一氧化碳中毒、溶血尿毒综合征、渗透性脱髓鞘和脑炎。慢性情况包括遗传性（“先天性代谢缺陷”、亨廷顿病和脱髓鞘疾病）或后天性（急性疾病的后遗症）情况，这些情况代表基底神经节内异常的生化或结构过程。消除急性病因几乎没有改善的希望。识别慢性疾病对于咨询目的很重要，因为这些疾病大多具有特定的遗传模式。
