Centre of Excellence for Neuropsychiatry, Vincent van Gogh Institute for Psychiatry, P.O. Box 5, 5800 AA, Venray, The Netherlands.
Donders Institute for Brain, Cognition and Behaviour, Radboud University, Nijmegen, The Netherlands.
J Clin Psychol Med Settings. 2020 Jun;27(2):256-267. doi: 10.1007/s10880-019-09659-7.
This is the first controlled study regarding personality and psychopathology in adults with Noonan syndrome (NS). Anxiety, depression, alexithymia and symptoms of Attention Deficit-Hyperactivity Disorder and Autism Spectrum Disorder, have been previously described in NS. More information regarding personality and psychopathology in NS could improve mental health care for this population. Therefore, scores on the Minnesota Multiphasic Personality Inventory-2-Restructured Form (MMPI-2-RF), a widely used self-report questionnaire of personality and psychopathology, were compared between patients with NS (n = 18) and matched, healthy controls (n = 18). Furthermore, correlations between MMPI-2-RF scores and alexithymia, measured by the Toronto Alexithymia Scale-20, were investigated. Patients with NS showed significantly higher scores, with medium effect sizes, on MMPI-2-RF scales reflecting infrequent responses (F-r), somatic and cognitive complaints (FBS-r and RBS-r), internalizing problems (EID), demoralization (RCd) and introversion (INTR-r), although the overall profile in both groups was within the non-clinical range. Alexithymia correlated with internalizing problems and negative emotionality in the patient group. In conclusion, patients with NS showed higher levels of introversion, which may predispose them to internalizing problems. These problems were indeed more frequent in patients with NS, especially higher levels of demoralization. Patients may benefit from psychological interventions aimed to decrease internalizing problems, introversion and alexithymia.
这是第一项关于努南综合征（Noonan syndrome，NS）成人的人格和精神病理学的对照研究。焦虑、抑郁、述情障碍以及注意力缺陷多动障碍和自闭症谱系障碍的症状此前已在 NS 中被描述过。更多关于 NS 人格和精神病理学的信息可以改善该人群的心理健康护理。因此，我们比较了 NS 患者（n=18）和匹配的健康对照组（n=18）在明尼苏达多相人格问卷-2 修订版（MMPI-2-RF）上的得分，这是一种广泛使用的人格和精神病理学自评问卷。此外，还研究了 MMPI-2-RF 得分与多伦多述情障碍量表-20 （Toronto Alexithymia Scale-20）测量的述情障碍之间的相关性。NS 患者在反映罕见反应（F-r）、躯体和认知抱怨（FBS-r 和 RBS-r）、内化问题（EID）、沮丧（RCd）和内向（INTR-r）的 MMPI-2-RF 量表上的得分显著更高，具有中等效应大小，尽管两组的总体表现都在非临床范围内。述情障碍与患者组的内化问题和负性情绪有关。总之，NS 患者表现出更高水平的内向性，这可能使他们易患内化问题。这些问题在 NS 患者中确实更为常见，尤其是沮丧水平更高。患者可能受益于旨在减少内化问题、内向性和述情障碍的心理干预。
