Type and Strength of Food Preferences of Individuals With Prader‐willi Syndrome*

Food-related Neural Circuitry in Prader-Willi Syndrome: Response to High- Versus Low-calorie Foods

“…Although our results do not indicate significant preference to high-calorie foods compared to low-calorie foods for participants with PWS, earlier research has shown that individuals with PWS have a preference for sweet foods relative to other foods (Taylor and Caldwell 1985). Our participants with PWS rated highand low-calorie foods equally high with mean ratings falling under the 'like very much' category.…”

“…High-calorie foods activated some regions more than low-calorie foods, including the hypothalamus, thalamus, and the medial and dorsolateral PFC, the latter of which are thought to be involved in inhibition of emotion and regulation of appetitive drives. Despite early perceptions that individuals with PWS eat indiscriminately, collective research with this population has shown that when quantity is equated, individuals with PWS show clear preference for foods based on their taste and macronutrient content, typically favoring tasty high-fat or carbohydrate foods to foods with lower caloric values (Taylor and Caldwell 1985;Fieldstone et al 1997;Glover et al 1996;Joseph et al 2002). Recently, Hinton et al (2006a) examined the role of food preference and incentive motivation on food-related neural substrates.…”

Management of Prader-Willi Syndrome

“…In 1993, Holm, Cassidy, Butler, and others 85 developed consensual diagnostic criteria to assist in the diagnosis of PWS using major and minor features and established a scoring system for patients presenting with features seen in this syndrome. The scoring system consisted of three categories (major, minor, and supportive criteria) and scoring was based on a point system.…”

“…Obesity or, more specifi cally, overweight has also been postulated to contribute to restrictive lung disease in PWS, as has been described in morbidly overweight individuals without PWS. 137 However, data suggest that respiratory muscle hypotonia may be a more important contributory factor, 85,156 especially since respiratory problems can be observed without excess body weight, as in neonates with PWS.…”

“…The relative lack of attention to such testing may, in part, be due to the usual need for active cooperation from the patient and consequent perceived diffi culties with PWS. Nonetheless, modifi ed pulmonary function testing data in children with PWS have been reported 85,131 with no signifi cant reports of problems with test feasibility.…”