Understanding severe disruptive behaviors in adolescents with fragile X syndrome

了解患有脆性 X 综合征的青少年的严重破坏性行为

• 批准号: 8512266
• 负责人: Scott S Hall
• 金额: $ 19.63万
• 依托单位: STANFORD UNIVERSITY
• 依托单位国家: 美国
• 财政年份: 2013
• 资助国家: 美国
• 起止时间: 2013-04-01 至 2015-03-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/8512266
• 关键词: Adolescent; Age; Aggressive behavior; Anxiety; Arousal; Autistic Disorder; Autonomic nervous system; Behavior; Behavior Therapy; Behavioral; Behavioral Mechanisms; Biological; Caring; Characteristics; Child; Complex; Consultations; Data; Development; Diagnosis; Distress; Education; Environmental Risk Factor; Exhibits; Family; Family Caregiver; Fragile X Syndrome; Frequencies; Future; Heart Rate; Hydrocortisone; Individual; Inherited; Intellectual functioning disability; Intervention; Investigation; Laboratories; Measures; Methodology; Negative Reinforcements; Parents; Participant; Pharmacological Treatment; Physiological; Population; Positive Reinforcements; Prevalence; Procedures; Process; Provider; Quality of life; Research; Salivary; Self-Injurious Behavior; Severities; Siblings; Sinus Arrhythmia; Specificity; Symptoms; Techniques; Training; Travel; Universities; aged; base; behavior influence; cohort; comparison group; developmental disease; effective intervention; environmental stressor; experience; heart rate variability; improved; innovation; male; public health relevance; respiratory; response; screening; social; tool

DESCRIPTION (provided by applicant): Adolescents diagnosed with fragile X syndrome (FXS), the leading known cause of inherited intellectual disability, commonly engage in severe disruptive behaviors (i.e., self-injury and aggression) that can significantly impact the individua's educational progress and functioning. However, the biological and behavioral mechanisms underlying these pathological behaviors are very poorly understood. In this proposal, we aim to examine the extent to which autonomic nervous system arousal interacts with environmental factors (positive and negative reinforcement processes) to exacerbate and maintain self-injury/aggression in FXS. Screening of potential participants will be conducted using the Functional Analysis Screening Tool(c) which will allow us establish the prevalence, frequency, severity, and circumstances surrounding the occurrence of self- injury/aggression in FXS. We will also screen individuals with intellectual disability (ID) who do not have FXS. From the screening, 30 individuals with FXS and 30 ID controls will be selected to travel to Stanford University for a 3-day assessment of their self-injury/aggression. These participants will be aged 11 to 18 years and engage in self-injury/aggression with moderate to severe intensity on a daily basis. Participants with FXS will be matched to ID control participants with respect to age, functioning level, and degree of autistic symptom severity. This comparison group will allow us to determine whether the behavioral characteristics of FXS are specific to FXS, or characteristic of individuals with ID in general. Each subject will undergo a Functional Analysis, conducted by a Board Certified Behavior Analyst, to identify the environmental factors maintaining the child's self-injury/aggression. The Functional Analysis will include both standard and "FXS-specific" conditions (e.g. transitions and social demands). To examine the influence of physiological factors, we will measure physiological responses and salivary cortisol levels both at Baseline and during the Functional Analysis. The results of this project will directly help individuals with FXS and their families, as well as greatly advance the understanding of self-injury/aggression in FXS, providing sorely needed empirical data to inform interventions in the future for both FXS and other individuals with ID. The project will also significantly improve our understanding of the complex interplay between physiological and environmental factors in co-morbid behaviors in individuals with FXS.

描述(由申请人提供)：被诊断为脆性X综合征(FXS)的青少年通常会从事严重的破坏性行为(即自残和攻击性)，这可能会显著影响个人的教育进步和功能。然而，这些病理行为背后的生物学和行为机制却知之甚少。在这项建议中，我们旨在研究自主神经系统唤醒与环境因素(积极和消极强化过程)相互作用的程度，以加剧和维持FXS的自我伤害/攻击。对潜在参与者的筛查将使用功能分析筛查工具(C)进行，该工具将使我们能够确定FXS中发生自我伤害/攻击的流行率、频率、严重性和情况。我们还将对没有FXS的智障人士(ID)进行筛查。从筛查中，30名患有FXS和30名ID控制的人将被选到斯坦福大学接受为期3天的自我伤害/攻击性评估。这些参与者的年龄在11岁到18岁之间，每天都会进行中等到严重强度的自我伤害/攻击。患有FXS的参与者将在年龄、功能水平和自闭症症状严重程度方面与ID对照组参与者进行匹配。这个比较组将使我们能够确定FXS的行为特征是FXS特有的，还是具有ID的个体的一般特征。每个受试者都将接受由委员会认证的行为分析员进行的功能分析，以确定维持儿童自我伤害/攻击性的环境因素。功能分析将包括标准条件和“FXS特定”条件(例如，过渡和社会需求)。为了检验生理因素的影响，我们将在基线和功能分析期间测量生理反应和唾液皮质醇水平。该项目的成果将直接帮助个人 FXS及其家人，并极大地促进了对FXS中自我伤害/攻击的理解，提供了急需的经验数据，为FXS和其他ID患者未来的干预提供信息。该项目还将显著提高我们对FXS和其他ID患者的理解 FXS患者共病行为中生理和环境因素之间复杂的相互作用。