Cognitive outcome in urea cycle disorders

被引：66
|
作者：
Gropman, AL [1 ]
Batshaw, ML [1 ]
机构：
[1] George Washington Univ, Sch Med & Hlth Sci, Childrens Res Inst, Childrens Natl Med Ctr,Dept Pediat, Washington, DC 20010 USA
来源：
MOLECULAR GENETICS AND METABOLISM | 2004年 / 81卷
关键词：
D O I：
10.1016/j.ymgme.2003.11.016
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
Despite treatment, cognitive and motor deficits are common in individuals with inherited urea cycle disorders. However, the extent to which the deficits involve specific cognitive or sensorimotor domains is unknown. Furthermore, little is known about the neurochemical basis of cognitive impairment in these disorders. This paper reviews studies of cognitive and motor dysfunction in urea cycle disorders, and discusses potential venues for investigation of the underlying neural basis that may elucidate these defects. Such methods of investigation may serve as a model for studying the relationship between genes, biochemical markers, brain function, and behavior in other metabolic diseases. (C) 2004 Elsevier Inc. All rights reserved.
引用
收藏
页码：S58 / S62
页数：5
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