基础医学与临床 ›› 2017, Vol. 37 ›› Issue (1): 99-102.

• 临床园地 • 上一篇    下一篇

Dyke–Davidoff–Masson 综合征1例

梁帅,窦万臣   

  1. 北京协和医院
  • 收稿日期:2016-06-06 修回日期:2016-10-28 出版日期:2017-01-05 发布日期:2016-12-30
  • 通讯作者: 窦万臣 E-mail:douwanchen@126.com

A case report of Dyke–Davidoff–Masson syndrome

,Wan-Chen DOU   

  • Received:2016-06-06 Revised:2016-10-28 Online:2017-01-05 Published:2016-12-30
  • Contact: Wan-Chen DOU E-mail:douwanchen@126.com

摘要: Dyke–Davidoff–Masson 综合征 (DDMS)是一种罕见癫痫综合征,以一侧半球萎缩、同侧颅骨代偿性改变和对侧偏瘫为特点,本文介绍一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。

关键词: Dyke–Davidoff–Masson 综合征, 半球萎缩, 癫痫, 多脑叶离断术

Abstract: 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 satisfactorily controlled in the following up of one year.

Key words: Dyke–Davidoff–Masson syndrome, cerebral hemiatrophy, seizure, multi-lobe disconnection

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