《中国康复理论与实践》 ›› 2014, Vol. 20 ›› Issue (5): 451-454.

• 论文 • 上一篇    下一篇

Duchenne型肌营养不良认知障碍机制研究进展

付雅,吴士文   

  1. 安徽医科大学武警总医院临床学院,北京市100039。
  • 收稿日期:2013-11-04 修回日期:2014-01-10 出版日期:2014-05-25 发布日期:2014-05-25
  • 通讯作者: 吴士文

Mechanisms of Cognitive Impairment of Duchenne's Muscular Dystrophy (review)

FU Ya, WU Shi-wen.   

  1. Clinical College of General Hospital of Armed Police Force, Anhui Medical University, Beijing 100039, China
  • Received:2013-11-04 Revised:2014-01-10 Published:2014-05-25 Online:2014-05-25

摘要: Duchenne 型肌营养不良是一种常见的X染色体连锁隐性遗传神经肌肉病,并常伴有认知障碍。目前关于其认知障碍机制研究较多,主要集中在抗肌萎缩蛋白亚型缺乏、中枢神经系统发育异常、突触功能改变、血脑屏障改变、大脑代谢改变及小脑功能异常等。

关键词: Duchenne型肌营养不良, 认知障碍, 抗肌萎缩蛋白, 突触, 血脑屏障, 代谢, 小脑, 综述

Abstract: Duchenne's muscular dystrophy (DMD) is a common X-linked recessive neuromuscular disorder that associated with a spectrum of genetically based cognitive and behavioral disabilities. Several mechanisms have been discussed as the cause of cognitive impairment in patients with DMD: absence of different dystrophin isoforms, impairments in neurogenesis, changes of synaptic plasticity, blood-brain barrier alterations, brain metabolic differences, cerebellum disfunction, etc.

Key words: Duchenne's muscular dystrophy, cognitive impairment, dystrophin, synapse, blood-brain barrier, metabolism, cerebellum, review