Dyke-Davidoff-Masson综合征临床及影像特征分析

Clinical and imaging features of Dyke-Davidoff-Masson syndrome

目的分析Dyke-Davidoff-Masson综合征(DDMS)的临床表现与影像学特征。方法回顾性分析13例DDMS患者的临床及CT、MRI资料。12例行CT平扫,4例行MRI平扫,3例同时行CT及MRI平扫。结果后天性DDMS 8例,先天性DDMS 5例,临床表现为癫痫12例,偏瘫9例,肢体萎缩3例,智力低下及精神障碍各9例,面部不对称2例,性功能障碍1例,双侧耳廓畸形1例。DDMS分型:Ⅰ型6例,Ⅱ型4例,Ⅲ型3例。13例均见单侧大脑半球萎缩及同侧侧脑室扩大,中线结构向患侧移位12例,同侧基底神经节及大脑脚萎缩各10例,海马萎缩6例,交叉性小脑萎缩及双侧小脑萎缩各3例,脑软化9例,脑穿通畸形4例,胼胝体细小3例,脑裂畸形并透明隔缺如1例。13例均有不同程度同侧颅骨代偿性改变,表现为颅骨增厚、额窦及乳突过度气化。结论DDMS主要临床表现为癫痫、偏瘫、智力低下及精神障碍,典型影像征象为单侧大脑半球萎缩及同侧颅骨代偿性改变。

Objective To analyze the clinical and imaging features of Dyke-Davidoff-Masson syndrome(DDMS).Methods The clinical data,unenhanced CT(12)and MRI(4)of 13 patients with type I(6),II(4),and III(3),acquired(8)and congenital(5)DDMS were retrospectively analyzed.Three of the 13 patients underwent both CT and MRI.Results The clinical manifestations included epilepsy(12),hemiplegia(9),mental retardation(9),mental disorders(9),extremity atrophy(3),facial asymmetry(2),sexual dysfunction(1),and bilateral auricular malformation(1).The patients showed unilateral cerebral hemiatrophy and ipsilateral ventricle dilatation(13),ipsilateral midline structural shift(12),ipsilateral basal ganglia and cerebral peduncle atrophy(10),encephalomalacia(9),hippocampal atrophy(6),porencephaly(4),crossed cerebellar atrophy(3),bilateral cerebellar atrophy(3),corpus callosum hypoplasia(3),schizencephaly and agenesis of septum pellucidum(1).All 13 patients had varying degrees of ipsilateral compensatory cranial changes of calvarial thickening,excessive aeration of the frontal sinus and mastoid air cells.Conclusion The main clinical features of DDMS are epilepsy,hemiplegia,mental retardation and mental disorders.Unilateral cerebral hemisphere atrophy and ipsilateral compensatory cranial changes are typical imaging features.