【摘要】 目的研究先天性肌营养不良(congenital muscular dystrophies,CMD)的MRI特点。资料与方法对10例临床诊断为CMD的患儿行常规头部MRI,分析其脑白质及脑发育异常情况。脑干发育是否正常经与对照组比较后进行判断。结果10例中,1例MRI表现基本正常,其余9例均有不同程度的脑白质异常,侧脑室旁白质异常8例,皮层下白质异常9例。1例有新生儿缺氧缺血脑病(HIE)后遗改变。1例有侧脑室扩大。1例小脑发育不良。2例小脑可见多发囊性小病灶。6例脑干发育不良。结论CMD的颅脑MRI具有特征性,MRI能为CMD的诊断提供帮助。更多还原

【Abstract】 Objective To investigate the MRI features of congenital muscular dystrophy.Materials and Meth-ods 10 patients with clinical diagnosis of congenital muscular dystrophy underwent cranial routine MRI examination to detect the white matter and brain structure abnormalities. The size of brain stem was measured for patients and controls to compare between them.Results MR imaging revealed 9 patients with cerebral white matter abnormalities and 1 patient with unremarkable findings. 2 patients had cerebellar cysts and 6 patients had brain stem hypoplasia. The sequelae of HIE,lateral ventricular enlargement and cerebella hypoplasia were observed in 1 patient respectively.Conclusion Cranial MRI can reveal cerebral white matter abnormalities in patients with congenital muscular dystrophy,which may be helpful in the diagnosis of the congenital muscular dystrophy.更多还原