Dyke-Davidoff-Masson综合征临床及影像学特征分析  

Analysis of clinical and imaging features in patient with Dyke-Davidoff-Masson syndrome

作　　者：汤子建 谢朝邦 刘衡 张体江 TANG Zijian;XIE Chaobang;LIU Heng;ZHANG Tijiang(Department of Radiology,the Affiliated Hospital of Zunyi Medical University,Zunyi,Guizhou Province 563000,China)

机构地区：[1]遵义医科大学附属医院放射科,贵州遵义563000

出　　处：《实用放射学杂志》2023年第11期1751-1754,共4页Journal of Practical Radiology

摘　　要：目的探讨戴克-大卫杜夫-梅森(Dyke-Davidoff-Masson)综合征(DDMS)的临床特征及影像学表现。方法回顾性分析13例经临床证实的DDMS患者临床特点、CT和/或MRI表现。结果13例临床表现均有癫痫,9例出现对侧肢体偏瘫或无力,11例表现智力低下、学习困难、反应迟钝,2例出现面部不对称。13例影像表现均出现单侧大脑半球弥漫性皮质和皮质下萎缩(左侧大脑半球萎缩8例,右侧大脑半球萎缩5例),豆状核、尾状核、背侧丘脑萎缩4例,大脑脚萎缩6例,11例同侧侧脑室扩张(其中2例合并脑穿通畸形),中线结构向患侧移位5例;颅骨增厚12例,9例鼻窦扩大(额窦7例,筛窦2例),2例乳突扩大;1例伴脑颜面血管瘤病(亦称Sturge-Weber综合征),4例胼胝体形态细小(1例伴压部部分缺如)。结论DDMS特征性影像表现为单侧大脑半球萎缩伴同侧颅骨代偿性改变,结合癫痫、偏瘫及智力障碍等临床表现综合分析可以做出正确的诊断。Objective To explore the clinical and imaging characteristics in patient with Dyke-Davidoff-Masson syndrome(DDMS).Methods The clinical data,CT and MRI manifestations of 13 patients with clinically confirmed DDMS were analyzed retrospectively.Results Of 13 patients,all presented clinical manifestations of epilepsy,and there were 9 cases with hemiplegia or weakness of the contralateral limbs,11 cases with mental retardation,learning difficulties and unresponsiveness,2 cases with facial asymmetry.Imaging findings of all patients showed cortical and subcortical atrophy in unilateral cerebral hemisphere,including 8 cases with left cerebral hemisphere atrophy and 5 cases with right cerebral hemisphere atrophy.And there were 4 patients with atrophy in the lenticular nucleus,caudate nucleus or thalamus,6 cases with atrophy in the cerebral peduncle,11 cases with ipsilateral lateral ventricle dilatation(including 2 cases with lateral ventricular perforation malformation),5 cases with ipsilateral midline structure shift;12 cases with calvarial thickening,9 cases with hyperpneumatization of the paranasal sinus(including 7 cases in the frontal sinus and 2 cases in the ethmoid sinus),2 cases with hyperpneumatization of the mastoid sinus;1 case with Sturge-Weber syndrome,and 4 cases with corpus callosum dysplasia(including 1 case with partial deficiency in the splenium of corpus callosum).Conclusion The imaging characteristics of DDMS are unilateral cerebral hemisphere atrophy and ipsilateral compensatory cranial changes.Clinical findings such as epilepsy,hemiplegia and intellectual disability are comprehensively analyzed to make a definite diagnosis.

关 键 词：Dyke-Davidoff-Masson综合征 计算机体层成像 磁共振成像 

分 类 号：R742[医药卫生—神经病学与精神病学] R814.42[医药卫生—临床医学] R445.2