发作性睡病1型是一种以白天过度嗜睡为特征的嗜睡性中枢紊乱,猝倒和其他快速眼动睡眠相关的表现。神经生理学研究表明,由于边缘结构和相关区域的结构和功能变化,1型发作性睡病患者可能会出现情绪处理障碍。然而,唯一一项探索嗜睡症行为反应的研究没有发现识别情绪的能力受到损害,可能是由于补偿机制。本研究旨在通过调查与情感处理相关的行为障碍来填补文献中的这一空白,重点是高级社会认知技能,即心理理论,儿科嗜睡症1型患者。对22名1型嗜睡症儿童和青少年(6名女性;年龄范围:8.0-13.5)和22名年龄和性别相匹配的健康对照(6名女性;年龄范围:8.9-13.0)进行了神经心理学评估,以评估社会经济状况。言语能力,工作记忆,社会焦虑和心理理论通过口头任务(即奇怪的故事任务)和视觉任务(即无声的电影)。还评估了1型发作性睡病患者的疾病严重程度。患者表现出心理理论技能受损,通过口头(对照中位数=8;患者中位数=5;P=0.009)和视觉任务(对照中位数=8;患者中位数=6;P=0.003)进行评估,与健康对照相比。相关分析表明,言语和视觉心理理论与发作性睡病严重程度呈负相关(ρ=-0.45,P=0.035和ρ=-0.52,P=0.012)。和白天嗜睡(ρ=-0.48,P=0.025和ρ=-0.45,P=0.038)。我们的研究表明,患有1型发作性睡病的儿童和青少年的心理理论领域存在选择性损害。此外,我们的结果强调了症状严重程度和心理理论之间的联系,这表明较低的心理理论水平与较高的症状严重程度有关。Further,需要进行纵向研究来理清这种关系的方向,并消除嗜睡症严重程度受损的儿童心理理论或心理理论技能是否通过提供更大的避免猝倒的能力来调节嗜睡症症状的严重程度的歧义。
Narcolepsy type 1 is a central disorder of hypersomnolence characterized by excessive daytime sleepiness, cataplexy and other rapid eye movement sleep-related manifestations. Neurophysiological studies suggest that
narcolepsy type 1 patients may experience impairment in emotional processing due to structural and functional changes in limbic structures and associated areas. However, the only study exploring
narcolepsy behavioural responses found no impairment in the ability to recognize emotions, possibly due to compensatory mechanisms. The present study was designed to fill this gap in the literature by investigating the behavioural impairment related to emotional processing focusing on an advanced socio-cognitive skill, namely Theory of Mind, in paediatric narcolepsy type 1 patients. Twenty-two
narcolepsy type 1 children and adolescents (six female; age range: 8.0-13.5) and 22 healthy controls matched for age and sex (six female; age range: 8.9-13.0) underwent a neuropsychological evaluation to assess socio-economic status, verbal abilities, working memory, social anxiety and Theory of Mind via a verbal task (i.e. Strange Stories task) and a visual task (i.e. Silent Films).
Narcolepsy type 1 patients were also evaluated for disease severity. Patients exhibited impairment in Theory of Mind skills, as assessed both through both verbal (controls median = 8; patients median = 5; P = 0.009) and visual tasks (controls median = 8; patients median = 6; P = 0.003), compared to healthy controls. Correlation analyses showed that verbal and visual Theory of Mind was negatively related to narcolepsy severity (ρ = -0.45, P = 0.035 and ρ = -0.52, P = 0.012), and daytime sleepiness (ρ = -0.48, P = 0.025 and ρ = -0.45, P = 0.038). Our study shows a selective impairment in the Theory of Mind domain in children and adolescents with narcolepsy type 1. In addition, our results highlight a link between symptom severity and Theory of Mind, suggesting that lower Theory of Mind levels are associated with higher symptom severity. Further, longitudinal studies are needed to disentangle the direction of this relation and to disambiguate if narcolepsy severity impaired children\'s Theory of Mind or if Theory of Mind skills modulate the severity of narcolepsy symptoms by providing a greater ability to avoid cataplexy.