Roelofs Renée L, Wingbermühle Ellen, Kessels Roy P C, Egger Jos I M
Centre of Excellence for Neuropsychiatry, Vincent van Gogh Institute for Psychiatry, Venray, The Netherlands,
Donders Institute for Brain, Cognition and Behaviour, Radboud University, Nijmegen, The Netherlands,
Neuropsychiatr Dis Treat. 2019 Feb 26;15:611-626. doi: 10.2147/NDT.S179527. eCollection 2019.
Noonan syndrome (NS) is a genetic disorder that is associated with social cognitive problems. While treatment aimed at the improvement of social cognition is available for other neuropsychiatric disorders, no such interventions yet exist for NS patients. In this study, the development of the first social cognitive training for NS patients is described and its applicability and feasibility evaluated.
Eleven adult patients with NS participated in this controlled proof-of-principle study. Six patients were included in the treatment group and five in the control group. Neuropsychological testing was performed in both groups at baseline and posttreatment. Social cognition was a primary outcome measure and nonsocial cognition and psychopathology secondary outcome measures. Differences between pre- and posttest were investigated with Wilcoxon signed-rank tests, and a process evaluation was performed to aid interpretation of the results.
Both groups were comparable with regard to age, estimated intelligence, and baseline performance. Although no significant differences were found between pre- and posttest scores on primary and secondary outcome measures in either group, a medium-large effect size was found on emotion recognition in the treatment group. Also, the process evaluation demonstrated the feasibility of the training.
This first social cognitive training for adult patients with NS has proven to be feasible for this population and showed some encouraging results regarding emotion recognition, although the training protocol could be optimized. Further investigation is required using a randomized controlled design in a larger sample, in order to substantiate the overall effectiveness of the training.
努南综合征(NS)是一种与社会认知问题相关的遗传性疾病。虽然针对其他神经精神疾病有旨在改善社会认知的治疗方法,但NS患者尚无此类干预措施。在本研究中,描述了首个针对NS患者的社会认知训练的开发,并评估了其适用性和可行性。
11名成年NS患者参与了这项对照性原理验证研究。治疗组纳入6名患者,对照组纳入5名患者。两组在基线和治疗后均进行了神经心理学测试。社会认知是主要结局指标,非社会认知和精神病理学是次要结局指标。采用Wilcoxon符号秩检验研究前后测差异,并进行过程评估以辅助结果解释。
两组在年龄、估计智力和基线表现方面具有可比性。虽然两组在主要和次要结局指标的前后测分数上均未发现显著差异,但治疗组在情绪识别方面发现了中等至较大的效应量。此外,过程评估证明了训练的可行性。
首个针对成年NS患者的社会认知训练已证明对该人群可行,并且在情绪识别方面显示出一些令人鼓舞的结果,尽管训练方案可以优化。需要在更大样本中使用随机对照设计进行进一步研究,以证实训练的总体有效性。