Computer-Based Cognitive Remediation in Adolescents with VCFS

描述（由申请人提供）：本申请响应RFA - MH - 09 - 021，提出研究计算机认知修复程序对青少年速心面综合征（VCFS）的可行性和临床应用。VCFS由染色体22q11.2上的微缺失引起，影响1:40 000个体，与特定的神经认知缺陷、行为障碍以及高达25%的老年青少年和年轻人的精神分裂症有关。二十年的研究已经确定，患有VCFS的年轻人在注意力、工作记忆和执行功能方面表现出缺陷。关键是我们现在设计针对这些缺陷的干预计划，以改善患有这种疾病的年轻人的功能，并有可能缓和严重精神症状的发作。在本应用中，我们建议采用先前验证的、计算机化的认知修复程序，重点关注青少年VCFS的注意力、执行功能和记忆，并利用标准化的神经心理学工具、适应功能测量和虚拟现实环境来评估修复程序的临床效用。由于在任何给定的大都市地区，患有VCFS的年轻人都相对较少，因此我们提出了一种高度创新的方法，即我们在锡拉丘兹的“认知教练”将利用高速互联网连接和视频会议软件，在年轻人自己的家中管理这个补救计划。该项目的R21阶段将侧重于a)调整修复软件以供患有VCFS的青少年使用，b)评估“远程”管理修复软件的耐受性和可行性，以及c)开发/试用虚拟现实评估软件，以纳入我们的干预前和干预后评估。该项目的R33阶段将评估认知补救方案的临床效用。采用交叉治疗设计，我们将20名患有VCFS的青少年（12 - 16岁）带到我们的医疗中心进行四项评估：基线时、9个月时、18个月时和24个月时。“远程”认知干预本身将在9到18个月的评估期间进行。为了确保我们的干预措施的生态有效性，我们的评估将包括标准化的神经心理学测试和我们将在项目的R21阶段开发的虚拟现实环境背景下的评估。该项目是实施一项影响深远、有效的干预方案的初步步骤，该方案有可能改善VCFS患者的日常生活，培养更独立的成年轨迹，从而减少这种疾病对家庭和社会造成的损失。

DESCRIPTION (provided by applicant): This application, in response to RFA - MH - 09 - 021, proposes to investigate the feasibility and clinical utility of a computerized cognitive remediation program for adolescents with velo-cardio-facial syndrome (VCFS). VCFS, caused by a microdeletion on chromosome 22q11.2 and affecting 1:4000 individuals, is associated with specific neurocognitive deficits, behavioral disorders and, in up to 25% of older adolescents and young adults, schizophrenia. Two decades of research has firmly established that youth with VCFS exhibit deficits in attention, working memory and executive function. It is critical that we now design intervention programs that target these deficits, in order to improve the functioning of youth with this disorder and potentially, to moderate the onset of severe psychiatric symptomatology. In this application, we propose to adapt a previously validated, computerized, cognitive remediation program, focused on attention, executive function, and memory, for adolescents with VCFS, and to evaluate the clinical utility of the remediation program, utilizing standardized neuropsychological instruments, measures of adaptive functioning and virtual reality environments. Since relatively few youth with VCFS live within any given metropolitan area, we are proposing a highly innovative approach whereby our Syracuse-based "cognitive coach" will utilize high-speed internet connections and videoconferencing software to administer this remediation program to youth in their own homes. The R21 phase of the project will focus on a) adapting the remediation software for use with adolescents with VCFS, b) evaluating the tolerability and feasibility of administering the remediation software "remotely", and c) developing / piloting virtual reality assessment software to include in our pre- and post-intervention assessments. The R33 phase of the project will evaluate the clinical utility of the cognitive remediation program. Using a crossover treatment design, we will bring 20 adolescents (ages 12 - 16 years) with VCFS to our medical center for four assessments: at baseline, 9 months, 18 months, and 24 months. The "remote" cognitive intervention itself will take place between the 9 and 18 month assessments. In order to ensure the ecological validity of our intervention, our assessments will consist of both standardized neuropsychological tests and assessments within the context of the virtual reality environments that we will develop during the R21 phase of the project. This project constitutes the initial steps toward implementing a far-reaching, effective intervention protocol that, potentially, will improve the daily lives of individuals with VCFS and foster a more independent trajectory to adulthood, thereby reducing the toll that this disorder takes on families and on society.