儿童白质消融性脑白质病的MRI表现  

MRI findings in children with vanishing white matter disease

作　　者：胡建敏[1] 张海华[2] 肖江喜[1] 吴晔[2] 谢晟[3] 王静敏[2] 

机构地区：[1]北京大学第一医院医学影像科,100034 [2]北京大学第一医院儿科,100034 [3]中日友好医院放射科

出　　处：《中华放射学杂志》2013年第10期908-911,共4页Chinese Journal of Radiology

摘　　要：目的 探讨白质消融性脑白质病（VWM）的MRI特征表现.方法 回顾性分析2008年至2013年间经本院儿科基因确诊为VWM的10例患儿的临床及MR资料.由2名资深的神经放射医师进行阅片,对白质病变部位（大脑、小脑、脑干、胼胝体及内外囊）、范围、信号特点、有无囊变、脑萎缩及基底节受累等进行分析.结果 10例大脑中央白质均弥漫受累,9例部分皮层下白质受累,7例伴U形纤维受累；7例伴有囊变；8例内囊后肢受累,7例外囊受累,8例胼胝体内缘（透明隔缘）受累.2例丘脑和苍白球出现异常信号,6例脑干白质受累,7例小脑白质受累.结论 大脑中央白质出现弥漫对称的异常信号伴囊变是VWM特征性的MRI表现,对该病的诊断具有重要价值.Objective To describe the MRI features of vanishing white matter disease （VWM）.Methods Ten patients diagnosed as VWM between 2008-2013 were enrolled,with the mean age of（40 ±23）months.The clinical materials and MR images of them were reviewed retrospectively.The findings of MRI were analyzed by two experienced neuroradiologists in this study,including the location （cerebrum,cerebellum,brainstem,corpus callosum and internal/external capsule）,size and signal intensity of the lesions,as well as the presence of cystic degeneration,cerebrum atrophy and the abnormal signal of the basal nuclei.Results MRI of brain typically showed extensive abnormal signal in the cerebral central white matter （10/10） and the focal lesion in subcortical white matter （9/10）.The involvement of U-fibers was present in 7 cases.Cystic degeneration of the affected white matter was found in 7 cases.Abnormal signal also appeared in the white matter of posterior limb of the internal capsule （8/10）,the external capsule （7/10）,the inner rim （the callosal-septal interface） of the corpus callosum （8/10）.The lesions of the thalamus and globus pallidus were detected in 2 cases.The white matter of the brainstem and cerebellum were involved in 6 and 7 cases respectively.Conclusion VWM typically shows a diffuse and symmetrical abnormal signal in the cerebral white matter with cystic degeneration on MRI,which is usually diagnostic.

关 键 词：脑白质病 进行性多灶性 磁共振成像 

分 类 号：R445.2[医药卫生—影像医学与核医学]