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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。
Objective To study the MRI features of congenital muscular dystrophies (CMD). Materials and Methods Ten patients with clinically diagnosed CMD underwent conventional head MRI and analyzed their white matter and brain dysplasia. Brainstem development is normal after comparison with the control group to judge. Results Of the 10 cases, the MRI findings were normal in 1 case. The other 9 cases had different degrees of white matter abnormalities, 8 cases of abnormal white matter in the lateral ventricle and 9 cases of abnormal cortical white matter. A case of neonatal hypoxic-ischemic encephalopathy (HIE) after the change. One case had lateral ventricle enlargement. One case of cerebellar dysplasia. 2 cases of cerebellum can be seen multiple cystic small lesions. Six cases of brainstem dysplasia. Conclusion The brain MRI of CMD is characteristic. MRI can help to diagnose CMD.