摘要
目的探讨甲基丙二酸血症(MMA)患儿颅脑常规MRI特点及其临床应用价值。资料与方法对13例MMA患儿的常规MRI图像进行回顾性分析。全部病例均经尿有机酸分析确诊。结果MRI主要表现为脑发育延迟和脑组织受损。其中,脑沟、脑池增宽者12例,合并侧脑室增宽者9例;脑白质容积减少者8例,脑白质髓鞘化延迟7例;胼胝体形态幼稚6例,另有1例胼胝体压部信号异常;苍白球显示长T1、长T2信号者1例。结论颅脑常规MRI可反映MMA对脑发育的影响和脑损伤,有助于了解病情和评估预后。
Objective To discuss the conventional brain MR findings and its role in evaluation of methylmalonic acidemia （MMA） in children. Materials and Methods MR data of 13 patients with MMA confirmed by analysis of urine were reviewed retrospectively. Results Brain conventional MRI findings mainly reflected cerebral development retardation and injuries. The presentations included widening of sulci and cisterns （12 cases）, lateral ventriculomegaly （9 cases）, bulk loss in the white matter （8 cases）, and immature form of corpus callosum （6 cases）. In addition, abnormal signal intensity was found in white matter （7 cases）, splenium of the corpus callosum （ 1 case）, and bilateral globus pallidus （ 1 case） respectively. Conclusion Brain conventional MR can reflect cerebral development retardation and injuries in children with MMA, it is helpful in assessing the status and prognosis of the disease.
出处
《临床放射学杂志》
CSCD
北大核心
2006年第12期1143-1146,共4页
Journal of Clinical Radiology
关键词
甲基丙二酸血症
儿童
磁共振成像
Methylmalonic acidemia Children Magnetic resonance imaging
