Luu Skylar, Province Haley, Berry-Kravis Elizabeth, Hagerman Randi, Hessl David, Vaidya Dhananjay, Lozano Reymundo, Rosselot Hilary, Erickson Craig, Kaufmann Walter E, Budimirovic Dejan B
Albany Medical Center, Albany Medical College, 43 New Scotland Ave, Albany, NY 12208, USA.
Feinberg School of Medicine, Northwestern University, 420 E. Superior St, Chicago, IL 60611, USA.
Brain Sci. 2020 Sep 11;10(9):629. doi: 10.3390/brainsci10090629.
Fragile X syndrome (FXS) is the leading cause of inherited intellectual disability and autism spectrum disorder. Individuals with FXS often present with a wide range of cognitive deficits and problem behaviors. Educational, behavioral and pharmacological interventions are used to manage these and other complex issues affecting individuals with FXS. Despite the success of preclinical models and early-phase drug clinical studies in FXS, large-scale randomized-controlled trials have failed to meet primary endpoints. Currently, no targeted or disease-modifying treatments for FXS have received regulatory approval. Here, we examined the placebo response in FXS clinical trials conducted between 2006 and 2018. Specifically, we performed a meta-analysis of placebo-treated groups in eight double-blind, randomized controlled trials. Placebo groups demonstrated significant improvements on caregiver-rated efficacy endpoints, which were greater in adolescents and adults than in children. Among the latter measures, the Visual Analog Scale scores displayed the greatest improvements, whereas the positive effects on the Vineland-II Adaptive Behavior Composite and the Aberrant Behavior Checklist-Community/fragile X version were statistically significant in both children and adolescents/adults. Although the Clinical Global Impression scale Improvement appears to have exhibited a substantial placebo effect in multiple clinical trials in FXS, limited data availability for meta-analysis, prevented us from drawing conclusions. No placebo-related improvements were observed in performance-rated measures. These findings raise substantial concerns about placebo effects in outcome measures commonly used in the randomized-controlled trials in FXS and suggest several potential improvements in the study design and implementation of such trials. Considering the small number of trials available for this study, larger and more detailed follow up meta-analyses are needed. Meanwhile, efforts to improve the measurement properties of endpoints and rater training in drug trials in FXS should be prioritized.
脆性X综合征(FXS)是遗传性智力残疾和自闭症谱系障碍的主要病因。患有FXS的个体通常存在广泛的认知缺陷和问题行为。教育、行为和药物干预被用于管理影响FXS患者的这些及其他复杂问题。尽管在FXS的临床前模型和早期药物临床研究中取得了成功,但大规模随机对照试验未能达到主要终点。目前,尚无针对FXS的靶向或疾病修饰治疗获得监管批准。在此,我们研究了2006年至2018年间进行的FXS临床试验中的安慰剂反应。具体而言,我们对八项双盲随机对照试验中接受安慰剂治疗的组进行了荟萃分析。安慰剂组在照顾者评定的疗效终点上显示出显著改善,青少年和成人的改善程度大于儿童。在后者的测量指标中,视觉模拟量表得分改善最大,而对文兰适应性行为量表第二版和异常行为检查表社区/脆性X版在儿童和青少年/成人中均有统计学意义的积极影响。尽管临床总体印象量表改善在FXS的多项临床试验中似乎表现出显著的安慰剂效应,但荟萃分析的数据可用性有限,使我们无法得出结论。在性能评定指标中未观察到与安慰剂相关的改善。这些发现引发了对FXS随机对照试验中常用结局指标的安慰剂效应的重大担忧,并提示了此类试验在研究设计和实施方面的一些潜在改进。考虑到本研究可用的试验数量较少,需要进行更大规模和更详细的后续荟萃分析。同时,应优先努力改善FXS药物试验中终点指标的测量特性和评定者培训。