Clinical manifestations and advances in hereditary research of alternating hemiplegia of childhood
10.3760/cma.j.issn.1673-4408.2014.03.003
- VernacularTitle:儿童交替性偏瘫的临床与遗传研究进展
- Author:
Shan LI
- Publication Type:Journal Article
- Keywords:
Alternating hemiplegia of childhood;
ATP1 A3;
Abnormal ocular movements;
Dystonia
- From:
International Journal of Pediatrics
2014;41(3):229-232
- CountryChina
- Language:Chinese
-
Abstract:
Alternating hemiplegia of childhood(AHC) is a hereditary disease characterized by hemiplegia spells,abnormal eye movements,dystonia and cognitive impairment.There are three phases of the disease.Each phase has its unique clinical symptoms:phase 1-abnormal eye movements and dystonia;phase 2-hemiplegia spells; phase 3-permanent cognitive impairment.The severity of cognitive impairment depends on the time of onset of hemiplegia spells:the earlier the onset is,the worse outcome will be.No effective treatment has been established.Thetreatments currently include:avoiding predisposing factors and taking drugs such as flunarizine to prevent hemiplegia attacks,in the inter-ictal stage;and sedation during hemiplegia attacks.According to the latest research,AHC is caused by the de novo mutation of gene ATP1A3.
