摘要
目的 探讨粘多糖病的头颅CT表现和临床的联系。方法 收集我院收治的7例粘多糖贮积症患儿的临床和CT资料。患儿均为男性，年龄范围为1岁1个月一10岁，其中PMSⅠ型4例、Ⅱ型1例和Ⅳ型2例。7例均进行头颅CT平扫，其中4例曾进行普通X线平片检查。结果 头颅CT平扫显示脑室扩张（6例）、脑外间隙（包括脑池和蛛网膜下腔）增宽（6例）和脑白质低密度灶（5例）。2例表现为脑白质密度普遍减低。结论 粘多糖病头颅CT表现主要为脑室扩张与脑外间隙增宽和脑白质低密度灶。其中脑室扩张和脑外问隙增宽可能与脑萎缩有关，并与患儿智力落后程度平行；脑白质内低密度可能为粘多糖在胶原细胞和血管间隙中沉着的结果。
Objective Was to determine the brain computed tomography （CT）abnormalities in a selected group of children with mueopolysaeeharidosis（MPS） types Ⅰ,Ⅱ and Ⅳ. We retrospectively assessed CT brain studies in 7 boys with MPS（type Ⅰ ：4, type Ⅱ ： 1 and type Ⅳ 2）, aged froml. 1 - 10 years. We evaluated abnormal density in the white matter（5 cases）, size of the supratentorial ventricles （6 cases）, and enlargement of the extra- brain spaces（6 cases）. Based on our results, we believe that with the exception of white matter abnormalities and brain atrophy, all other findings may be related to abnormal resorption of CSF, and the severity of hypodensity in white matter and brain atrophy were parallel to mental retardation.
出处
《中华临床医学杂志》
2005年第8期16-17,共2页
Chinese Journal of Clinical Practical Medicine
