STARTLE RESPONSE ANALYSIS OF FOOD-IMAGE PROCESSING IN PRADER-WILLI SYNDROME.

The Journal of rare disorders Pub Date : 2018-10-01
Alex Gabrielli, Albert B Poje, Ann Manzardo, Merlin G Butler
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Abstract

Background: Prader-Willi syndrome (PWS) is a complex genetic neurodevelopmental disorder with endocrine disturbances, hyperphagia and often life-threatening obesity as key features. We investigated emotional-processing of food and eating behavior in PWS using startle response-modulation. Startle eyeblink response is an involuntary reflex activated by the autonomic nervous system in response to sudden or disturbing auditory/visual stimuli which may be modulated by the emotional valence of concurrently viewed visual stimuli.

Methodology: Differences in affective modulation of startle reflex were recorded in 13 individuals with PWS versus 8 healthy controls when viewing standard neutral, negative, positive and food-derived images. Electromyogram (EMG) of the orbicularis oculi muscle was measured in response to binaural white noise before and after consumption of a standard 500 Kcal meal. Participants reported their perceived emotional valence for each image, pre- and post-meal, using a 1-10 Likert rating scale.

Results: Subjective ratings of food images and urge to eat were significantly higher in PWS than controls and did not significantly decline post-meal. Acoustic startle responding was detected in PWS but was significantly lower than control participants under all conditions. Startle responses to food images in PWS were attenuated relative to other picture types with potentially abnormal emotional modulation of responses to non-food images which contrasted self-reported picture ratings. A stable positive emotional valence to food images was observed pre- and post-feeding with a sustained urge to consume food in PWS.

Conclusions: Emotional processing measured using startle modulation in response to non-food images was abnormal in PWS which may reflect unique physiological attributes such as hypotonia and abnormal skin conductivity due to increased fat mass. Alternatively, disruption of autonomic or sympathetic nervous system functioning reported in PWS may impact on hunger and/or food drive states. Our findings parallel attentional/processing attributes of affective stimuli reported in autism spectrum disorder and support the feasibility of eyeblink startle modulation to assess food motivation in PWS and provide preliminary data to optimize methodological parameters.

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prader-willi综合征食物图像处理的惊吓反应分析。
背景:Prader-Willi综合征(PWS)是一种复杂的遗传性神经发育障碍,主要特征为内分泌紊乱、嗜食和危及生命的肥胖。我们利用惊吓反应调节研究了PWS患者对食物的情绪加工和进食行为。惊眨眼反应是由自主神经系统对突然或干扰的听觉/视觉刺激所激活的一种不自主反射,这种反应可能被同时观看的视觉刺激的情绪效价所调节。方法:记录了13名PWS患者与8名健康对照者在观看标准的中性、消极、积极和食物来源图像时惊吓反射情感调节的差异。在进食500千卡标准餐前后测量眼轮匝肌对双耳白噪声的肌电反应。参与者用1-10的李克特量表报告了他们对每张图片在餐前和餐后的感知情绪效价。结果:PWS患者对食物图像的主观评分和进食冲动明显高于对照组,餐后没有明显下降。在所有条件下,PWS均检测到声惊吓反应,但显著低于对照组。PWS对食物图像的惊吓反应相对于其他图像类型有所减弱,对非食物图像的反应可能存在异常情绪调节,这与自我报告的图像评级形成了对比。在进食前和进食后,观察到PWS患者对食物图像有稳定的积极情绪效价,并有持续的进食冲动。结论:通过惊吓调节测量的非食物图像的情绪加工在PWS中是异常的,这可能反映了由于脂肪量增加导致的低张力和皮肤传导异常等独特的生理属性。另外,PWS中报告的自主神经或交感神经系统功能的破坏可能会影响饥饿和/或食物驱动状态。我们的研究结果与自闭症谱系障碍中情感刺激的注意/加工属性相似,支持眨眼惊吓调节评估PWS食物动机的可行性,并为优化方法参数提供了初步数据。
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STARTLE RESPONSE ANALYSIS OF FOOD-IMAGE PROCESSING IN PRADER-WILLI SYNDROME. EVALUATION OF PLASMA SUBSTANCE P AND BETA-ENDORPHIN LEVELS IN CHILDREN WITH PRADER-WILLI SYNDROME. Taste perception and sensory sensitivity: Relationship to feeding problems in boys with Barth Syndrome. PHENOTYPIC VARIABILITY IN INDIVIDUALS WITH TYPE V OSTEOGENESIS IMPERFECTA WITH IDENTICAL IFITM5 MUTATIONS. Brain Vascular Malformation Consortium: Overview, Progress and Future Directions.
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