Baumgardner T L, Reiss A L, Freund L S, Abrams M T
Behavioral Neurogenetics and Neuroimaging Research Center, Kennedy Krieger Institute, Baltimore, MD 21205, USA.
Pediatrics. 1995 May;95(5):744-52.
A controlled clinical study was designed to identify the neurobehavioral profile that is specific to males with fragile X syndrome.
Thirty-one males with fragile X syndrome and 30 age and IQ-matched male controls were evaluated with instruments that assess multiple domains of adaptive functioning and problem behaviors. The Vineland Adaptive Behavior Scales and the Aberrant Behavior Checklist were selected for their dimensional scaling of behavioral ratings.
Parent and Teacher versions of the Aberrant Behavior Checklist demonstrated a profile of behaviors specific to males with fragile X syndrome characterized by significantly higher levels of hyperactivity, stereotypic movements, and unusual speech. The Vineland Adaptive Behavior Scales revealed no fragile X-specific profile of adaptive skills development.
The distinct pattern of aberrant behavior observed among males with fragile X emphasizes the importance of drawing subtype distinctions within the classification of individuals with mental retardation on the basis of underlying etiology. For clinical research, specifying the fragile X phenotype is a vital part in the effort to elucidate the neurodevelopmental pathways of normal behavior and psychopathology. Understanding the fragile X symptom pattern is essential for designing symptom-specific treatment interventions, as well as for research into the efficacy of interventions strategies.
设计一项对照临床研究，以确定脆性X综合征男性所特有的神经行为特征。
使用评估适应性功能和问题行为多个领域的工具，对31名脆性X综合征男性以及30名年龄和智商匹配的男性对照进行评估。选择文兰适应行为量表和异常行为清单是因为它们对行为评分进行维度缩放。
异常行为清单的家长版和教师版显示，脆性X综合征男性具有特定的行为特征，其多动、刻板动作和异常言语水平显著更高。文兰适应行为量表未显示出脆性X综合征特有的适应技能发展特征。
在脆性X综合征男性中观察到的独特异常行为模式强调了根据潜在病因在智力障碍个体分类中区分亚型的重要性。对于临床研究而言，明确脆性X综合征的表型是阐明正常行为和精神病理学神经发育途径的重要组成部分。了解脆性X综合征的症状模式对于设计针对特定症状的治疗干预措施以及研究干预策略的疗效至关重要。
