Dyke-Davidoff-Masson综合征1例

A case report of Dyke-Davidoff-Masson syndrome

Dyke-Davidoff-Masson综合征(DDMS)是一种罕见癫痫综合征,以1侧半球萎缩、同侧颅骨代偿性改变和对侧偏瘫为特点,本文介绍1例反复癫痫发作药物控制不佳案例,查体发现智力迟钝、面部不对称、右侧肢体轻偏瘫,MRI提示左侧大脑半球萎缩、颅骨增厚,正电子发射计算机断层显像(PET-CT)提示左侧大脑半球代谢减低,最终行多脑叶离断术缓解癫痫发作,术后随访1年,癫痫发作控制满意。

Dyke-Davidoff-Masson syndrome（ DDMS） is a rare epilepsy syndrome which is characterized by cerebral hemiatrophy with ipsilateral compensatory skull changes and contralateral hemiplegia recurrent. Here we reported a case of which the curative effect turned out to be unsatisfied after conservative treatment,physical examination revealed mental retardation,facial asymmetry,mild right hemiparesis,MRI scan of the head showed left cerebral hemiatrophy,calvaria thickening,PET-CT showed less functional left cerebral hemisphere. The patient finally underwent multi-lobe disconnection to relieve recurrent seizure,whose seizure was well controlled in the following up of one year.