Finucane B, Dirrigl K H, Simon E W
Elwyn Training and Research Institute, PA 19063, USA.
Am J Ment Retard. 2001 Jan;106(1):52-8. doi: 10.1352/0895-8017(2001)106<0052:COSIBI>2.0.CO;2.
A characteristic pattern of stereotypic and self-injurious behavior (SIB) distinguishes Smith-Magenis syndrome from many other genetic disorders. We examined the prevalence and severity of 11 specific types of SIB in 29 children and adults with Smith-Magenis syndrome. We confirmed the near universal presence of SIB in people with this disorder. The overall prevalence of SIB was found to increase with age as was the number of different types of SIB demonstrated by individuals with Smith-Magenis syndrome. The number of different types of SIB exhibited was also directly correlated with level of intellectual functioning. Our data suggest that with increasing age and ability levels, people with Smith-Magenis syndrome add to their repertoire of SIB from among a small number of specific behaviors.
刻板性自伤行为（SIB）的特征模式使史密斯-马吉尼斯综合征区别于许多其他遗传疾病。我们对29名患有史密斯-马吉尼斯综合征的儿童和成人的11种特定类型的SIB的患病率和严重程度进行了研究。我们证实了这种疾病患者中SIB几乎普遍存在。结果发现，SIB的总体患病率随年龄增长而增加，史密斯-马吉尼斯综合征患者表现出的不同类型SIB的数量也随年龄增长而增加。所表现出的不同类型SIB的数量也与智力功能水平直接相关。我们的数据表明，随着年龄和能力水平的增加，患有史密斯-马吉尼斯综合征的人会从少数特定行为中增加其SIB的种类。
