Effects of Topiramate in Adults With Prader-Willi Syndrome

Shapira, Nathan A.; Lessig, Mary; Lewis, Mark H.; Goodman, Wayne K.; Driscoll, Daniel J.

doi:10.1352/0895-8017(2004)109<301:eotiaw>2.0.co;2

Cited by 75 publications

(42 citation statements)

References 37 publications

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“…This was avoided by enhancing the appetite VAS with simple pictures of increasing amounts of the meal above the numeric scale. Satiety was assessed according to Shapira et al (2004), where the VAS was enhanced with cartoons of increasingly protruding bellies above the numeric scale ( Supplementary Fig. 1 Inter-and intra-assay CVs were <3.5%.…”

Section: Body Compositionmentioning

confidence: 99%

In adults with Prader–Willi syndrome, elevated ghrelin levels are more consistent with hyperphagia than high PYY and GLP-1 levels

et al. 2011

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ObjectivePrader-Willi syndrome (PWS) is a leading genetic cause of obesity, characterized by hyperphagia, endocrine and developmental disorders. It is suggested that the intense hyperphagia could stem, in part, from impaired gut hormone signaling. Previous studies produced conflicting results, being confounded by differences in body composition between PWS and control subjects. Design Fasting and postprandial gut hormone responses were investigated in a cross-sectional cohort study including 10 adult PWS, 12 obese subjects matched for percentage body fat and central abdominal fat, and 10 healthy normal weight subjects. Methods PYY [total], PYY , GLP-1[active] and ghrelin [total] were measured by ELISA or radioimmunoassay. Body composition was assessed by dual energy X-ray absorptiometry. Visual analog scales were used to assess hunger and satiety. Results In contrast to lean subjects (p < 0.05), PWS and obese subjects were similarly insulin resistant and had similar insulin levels. Ghrelin[total] levels were significantly higher in PWS compared to obese subjects before and during the meal (p < 0.05). PYY meal responses were higher in PWS than in lean subjects (p = 0.01), but not significantly different to obese (p = 0.08), with an additional non-significant trend in PYY[total] levels. There were no significant differences in self-reported satiety between groups, however PWS subjects reported more hunger throughout (p = 0.003), and exhibited a markedly reduced mealinduced suppression of hunger (p = 0.01) compared to lean or obese subjects. Conclusions Compared to adiposity-matched control subjects, hyperphagia in PWS is not related to a lower postprandial GLP-1 or PYY response. Elevated ghrelin levels in PWS are consistent with increased hunger and are unrelated to insulin levels.

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Section: Body Compositionmentioning

confidence: 99%

In adults with Prader–Willi syndrome, elevated ghrelin levels are more consistent with hyperphagia than high PYY and GLP-1 levels

et al. 2011

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“…Appetite-reducing drugs used in simple obesity have not shown wide-spread improvements, 102 and bariatric procedures have been associated with severe complications. 103,104 Growth hormone treatment has been shown to improve the fat-muscle mass ratio, 105 and may help to avoid the obesity for the younger generations of people with PWS, but has no effect on eating behaviour.…”

Section: Treatment Implicationsmentioning

confidence: 99%

Development of the eating behaviour in Prader–Willi Syndrome: advances in our understanding

2010

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Prader-Willi Syndrome (PWS) is a genetically determined neurodevelopmental disorder associated with mild to moderate intellectual disability, growth and sex-hormone deficiencies and a propensity to overeat that leads to severe obesity. The PWS phenotype changes from an early disinterest in food to an increasing pre-occupation with eating and a failure of the normal satiety response to food intake. The prevention of severe obesity is primarily through strict control of access to food and it is this aspect that most limits the independence of those with PWS. This review considers the eating disorder in PWS, specifically how the as yet uncertain genetics of the syndrome and the transition from the early to the later phenotype might account for the later hyperphagia. On the basis of behavioural and imaging studies, a failure of satiety and excessive activation of neural reward pathways have both been suggested. We speculate that the overeating behaviour, consequent upon one or other of the above, could either be due to a direct effect of the PWS genotype on the feeding pathways of the hypothalamus or a consequence of prenatal changes in the regulation of genes responsible for energy balance that sets a high satiation threshold. Understanding the overeating in PWS will lead to more focused and successful management and ultimately, treatment of this life-threatening behaviour. International Journal of Obesity (2011) IntroductionWe review the present state of knowledge on the eating disorder and potential resultant life-threatening obesity that is associated with Prader-Willi Syndrome (PWS), a genetically determined neurodevelopmental disorder affecting 1 in 22-25 000 live births.1-3 The aims of the review are: first, to consider our understanding of the eating disorder associated with PWS primarily from a behavioural and neuroscience perspective; second, to speculate as to the possible mechanisms that might directly or indirectly link the PWS genotype to the 'eating' phenotype; and third, to reflect on the implications for future research and for treatment. We discuss the major theories that could account for eating behaviour and whether any of these adequately explain the complex array of characteristics that exist in PWS and fit with what we know about the pathophysiology and the genetics of the disorder. The main discussion items include considering PWS as a disorder of satiety, as well as theories of hyper-responsive rewards systems and over-sensitivity to food stimuli, as has been proposed in general obesity. Further, we consider the importance of the prenatal environment and examine the possibility that people with PWS have an altered inner physical awareness, as shown by abnormal pain responses, and how this may alter the eating behaviour. MethodThis review has been carried out through a search of relevant articles primarily on the PubMed search engine (www. pubmed.gov) using 'eating behaviour Prader-Willi syndrome OR Prader Willi syndrome' as the search term. As the eating behaviour is such a key feature of t...

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“…O efeito do topiramato na perda de peso não foi tão evidente, mas todos os pacientes tiveram estabilização do ganho ponderal progressivo. Em outro estudo, essa droga não se mostrou eficaz na redução da ingestão calórica ou do apetite quando administrada por 8 semanas (36). Porém, outros estudos demonstraram efeito benéfico do topiramato em reduzir lesões provocadas por comportamento de automutilação em pacientes com SPW (36,37).…”

Section: Obesidade E Síndrome De Prader-williunclassified

Abordagem terapêutica da obesidade na Síndrome de Prader-Willi

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et al. 2007

Arq Bras Endocrinol Metab

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RESUMOA Síndrome de Prader-Willi (SPW) é uma doença complexa, multissistêmica, caracterizada por hipotonia, retardo mental, características dismórficas, hiperfagia e compulsão alimentar devido à disfunção hipotalâ-mica. SPW ocorre pela perda de função de genes localizados no cromossomo 15q11-13, região que sofre imprinting genômico. Obesidade é a principal causa de morbidade e mortalidade entre pacientes com SPW. O objetivo desta revisão é analisar as opções terapêuticas disponíveis para o tratamento da obesidade na SPW, incluindo a terapia farmacológica e o tratamento cirúrgico.

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Effects of Topiramate in Adults With Prader-Willi Syndrome

Cited by 75 publications

References 37 publications

In adults with Prader–Willi syndrome, elevated ghrelin levels are more consistent with hyperphagia than high PYY and GLP-1 levels

In adults with Prader–Willi syndrome, elevated ghrelin levels are more consistent with hyperphagia than high PYY and GLP-1 levels

Development of the eating behaviour in Prader–Willi Syndrome: advances in our understanding

Abordagem terapêutica da obesidade na Síndrome de Prader-Willi

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