Eye tracking as an objective measure of hyperphagia in children with <scp>Prader‐Willi</scp> syndrome

Key, Alexandra P.; Zengin-Bolatkale, Hatun; Dimitropoulos, Anastasia; Doernberg, Ellen

doi:10.1002/ajmg.a.61606

Cited by 5 publications

(3 citation statements)

References 35 publications

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“…Two recent reports of clinical trials for hyperphagia have reported baseline mean HQ-CT scores between 15 and 18.3 in obese adolescents and adults with PWS [41,42], scores that are much higher than the mean score (12.2) we found in obese individuals in our study. Another recent clinical study also reported higher baseline mean HQ-CT scores (around 18) in children with PWS aged 3-11 y [43]. The difference of research settings may explain the higher baseline scores in these clinical studies compared to the current study.…”

Section: Plos Onecontrasting

confidence: 41%

Characteristics and relationship between hyperphagia, anxiety, behavioral challenges and caregiver burden in Prader-Willi syndrome

et al. 2021

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Objectives Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by maladaptive behaviors, amongst which hyperphagia is a life-long concern for individuals with PWS and their caregivers. The current study examined the contribution of hyperphagia and other factors to caregiver burden across lifespan, in 204 caregivers of individuals with PWS living in the US, using the Zarit Burden Interview (ZBI) and the hyperphagia questionnaire (HQ-CT). Results We found a strong relationship between ZBI and HQ-CT especially in individuals with PWS older than 4 y and showed that HQ-CT scores of individuals with PWS is positively correlated with ZBI scores of their caregivers. The weight status of individuals with PWS was not associated with HQ-CT and ZBI scores, except for obese individuals who had significantly higher HQ-CT scores when compared to normal weight PWS individuals. We looked at PWS symptoms and care-related issues that impacted individuals and caregivers the most. We found that care-related tasks had the biggest negative impact on caregivers of children aged 0–4 y, whereas anxiety, temper tantrums, and oppositional behaviors of older individuals with PWS had the biggest impact on their caregivers concomitant with their high caregiver burden. Finally, we assessed the variability of HQ-CT and ZBI over 6 months in a subgroup of 83 participants. Overall, neither measure differed between 6 months and baseline. Most individual’s absolute HQ-CT score changes were between 0–2 units, whereas absolute ZBI score changes were between 0–6 points. Changes in the caregiver’s or individual’s life had little or no effect on HQ-CT and ZBI scores. Conclusions This study demonstrates a relationship between hyperphagia and caregiver burden and sheds light on predominant symptoms in children and adolescents that likely underly PWS caregiver burden. The stability and relationship between HQ-CT and ZBI support ZBI as an additional outcome measure in PWS clinical trials.

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Section: Plos Onecontrasting

confidence: 41%

Characteristics and relationship between hyperphagia, anxiety, behavioral challenges and caregiver burden in Prader-Willi syndrome

et al. 2021

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“…This observer-reported outcome measure, which aims to measure more objective food-related behavior, is based on behaviors over the past 2 weeks reported by a caregiver who is familiar with the daily activities of the person with PWS. Beyond self- or observer-reported assessment of hyperphagia in PWS, there is interest in the field for developing assessment tools that employ objective, direct measures of food interest, such as eye gaze or evoked response potentials [ 46 , 47 ].…”

Section: Hyperphagia In Pwsmentioning

confidence: 99%

Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium

Schwartz

Caixàs

Dimitropoulos

et al. 2021

J Neurodevelop Disord

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Prader-Willi syndrome (PWS) is a rare neurodevelopmental genetic disorder associated with a characteristic behavioral phenotype that includes severe hyperphagia and a variety of other behavioral challenges such as temper outbursts and anxiety. These behaviors have a significant and dramatic impact on the daily functioning and quality of life for the person with PWS and their families. To date, effective therapies addressing these behavioral challenges have proven elusive, but several potential treatments are on the horizon. However, a limiting factor for treatment studies in PWS is the lack of consensus in the field regarding how to best define and measure the complex and interrelated behavioral features of this syndrome. The International PWS Clinical Trials Consortium (PWS-CTC, www.pwsctc.org) includes expert PWS scientists, clinicians, and patient advocacy organization representatives focused on facilitating clinical trials in this rare disease. To address the above gap in the field, members of the PWS-CTC “Behavior Outcomes Working Group” sought to develop a unified understanding of the key behavioral features in PWS and build a consensus regarding their definition and description. The primary focus of this paper is to present consensus definitions and descriptions of key phenotypic PWS behaviors including hyperphagia, temper outbursts, anxiety, obsessive–compulsive behaviors, rigidity, and social cognition deficits. Patient vignettes are provided to illustrate the interrelatedness and impact of these behaviors. We also review some available assessment tools as well as new instruments in development which may be useful in measuring these behavioral features in PWS.

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“…However, findings concerning the association between genetic subtypes in PWS and food-related problems are inconclusive. For instance, higher food-related problems were found in individuals with microdeletion than those with mUPD ( 19 ) while Key et al ( 26 ) found a longer fixation on food stimuli using an eye tracking experimental procedure (a proxy measure of problematic food interest), in mUPD subtype than the deletion subgroup.…”

Section: Introductionmentioning

confidence: 99%

The Hyperphagia Questionnaire: Insights From a Multicentric Validation Study in Individuals With Prader Willi Syndrome

et al. 2022

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Background/ObjectivesThe present study aimed to validate the Italian version of the Hyperphagia Questionnaire (HQ), a 11-items questionnaire developed to assess hyperphagia in individuals with Prader-Willi syndrome (PWS). This is a complex neurodevelopmental disorder characterized by endocrine dysfunction, hypotonia, intellectual disability, psychiatric disorders and obesity.MethodsParents of 219 individuals with PWS (age range 3–54 years; Mage = 17.90; 108 Males), recruited in 12 hospitals in Italy responded to HQ during routine visits. In function of the level of analyses the sample was divided into two subgroups (<18> years) or into four age-subgroups (2.5–4.5; 4.5–8; 8–18; >18 years) corresponding to different clinical stages.ResultsConfirmatory factor analysis (CFA) confirmed the three hyperphagic subdimensions of the original structure (behavior, drive, and severity), but one item was dropped out, reducing the final version to 10 items. Using multi-group CFA, HQ showed satisfactory indexes of measurement invariance by age. Good indexes of internal consistency (Cronbach's alpha and McDonald's Omega coefficients) were found for each subdimension. The three hyperphagia subdimensions positively converged with other food-related measures: emotional overeating, food enjoyment, food responsiveness, and satiety responsiveness. A significant increase of all hyperphagic subdimensions was found across age groups. Higher hyperphagic levels were found in participants with higher body mass index. Hyperphagic drive differently increased in function of the interaction between age and underlying genetic mechanisms.ConclusionThe Italian version of the HQ is a psychometrically valid and reliable instrument for assessing hyperphagia in individuals with PWS. This tool may prove useful to evaluate the efficacy of pharmacologic and rehabilitative treatments.

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Eye tracking as an objective measure of hyperphagia in children with Prader‐Willi syndrome

Cited by 5 publications

References 35 publications

Characteristics and relationship between hyperphagia, anxiety, behavioral challenges and caregiver burden in Prader-Willi syndrome

Characteristics and relationship between hyperphagia, anxiety, behavioral challenges and caregiver burden in Prader-Willi syndrome

Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium

The Hyperphagia Questionnaire: Insights From a Multicentric Validation Study in Individuals With Prader Willi Syndrome

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