Abstract: Urea circle disorders are genetic metabolic diseases characterized by hyperammonemia. The main clinical manifestations are nervous system symptoms and liver injury，it can also be manifested as psychotic behavior abnormality，including mental retardation，insanity，autism spectrum disorder，depression，anxiety or mania.Lowering ammonia levels in the blood is a key step in improving the mental abnormalities of urea circle disorder. This article reviews the mechanism，clinical identification，progress in treatment measures at home and abroad of mental disorders caused by urea circle disorders. 

Key words: urea cycle disorders, hyperammonemia, mental disorder, behaviour disorder

摘要： 尿素循环障碍（urea cycle disorders，UCDs）是一类以血氨升高为主要特征的遗传代谢病，临床表现除神经系统症状及肝功能损害外，还可表现为智能损害、精神错乱、孤独症谱系障碍、抑郁、焦虑或躁狂等精神行为异常。降低血氨是改善UCDs精神异常的关键步骤。本章就UCDs导致精神异常的发生机制、临床识别及治疗措施国内外进展加以综述。

关键词: 尿素循环障碍, 高氨血症, 精神障碍, 行为异常