Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Review
  • Published:

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

Abstract

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.

This is a preview of subscription content, access via your institution

Access options

Rent or buy this article

Prices vary by article type

from$1.95

to$39.95

Prices may be subject to local taxes which are calculated during checkout

Similar content being viewed by others

References

  1. Whittington JE, Holland AJ, Webb T, Butler J, Clarke D, Boer H . Population prevalence and estimated birth incidence and mortality rate for people with Prader-Willi syndrome in one UK Health Region. J Med Genet 2001; 38: 792–798.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Smith A, Egan J, Ridley G, Haan E, Montgomery P, Williams K et al. Birth prevalence of Prader-Willi syndrome in Australia. Arch Dis Child 2003; 88: 263–264.

    CAS  PubMed  PubMed Central  Google Scholar 

  3. Vogels A, Van Den Ende J, Keymolen K, Mortier G, Devriendt K, Legius E et al. Minimum prevalence, birth incidence and cause of death for Prader-Willi syndrome in Flanders. Eur J Hum Genet 2004; 12: 238–240.

    CAS  PubMed  Google Scholar 

  4. Goldstone AP . Prader-Willi syndrome: advances in genetics, pathophysiology and treatment. Trends Endocrinol Metab 2004; 15: 12–20.

    CAS  PubMed  Google Scholar 

  5. Horsthemke B, Wagstaff J . Mechanisms of imprinting of the Prader-Willi/Angelman region. Am J Med Genet A 2008; 146A: 2041–2052.

    CAS  PubMed  Google Scholar 

  6. Holm VA, Cassidy SB, Butler MG, Hanchett JM, Greenswag LR, Whitman BY et al. Prader-Willi syndrome: consensus diagnostic criteria. Pediatrics 1993; 91: 398–402.

    CAS  PubMed  Google Scholar 

  7. Whittington J, Holland A, Webb T, Butler J, Clarke D, Boer H . Relationship between clinical and genetic diagnosis of Prader-Willi syndrome. J Med Genet 2002; 39: 926–932.

    CAS  PubMed  PubMed Central  Google Scholar 

  8. Cassidy SB, Driscoll DJ . Prader-Willi syndrome. Eur J Hum Genet 2009; 17: 3–13.

    CAS  PubMed  Google Scholar 

  9. Clarke DJ, Boer H, Whittington J, Holland A, Butler J, Webb T . Prader-Willi syndrome, compulsive and ritualistic behaviours: the first population-based survey. Br J Psychiatry 2002; 180: 358–362.

    CAS  PubMed  Google Scholar 

  10. Holland AJ, Whittington JE, Butler J, Webb T, Boer H, Clarke D . Behavioural phenotypes associated with specific genetic disorders: evidence from a population-based study of people with Prader-Willi syndrome. Psychol Med 2003; 33: 141–153.

    CAS  PubMed  Google Scholar 

  11. Greaves N, Prince E, Evans DW, Charman T . Repetitive and ritualistic behaviour in children with Prader-Willi syndrome and children with autism. J Intellect Disabil Res 2006; 50: 92–100.

    CAS  PubMed  Google Scholar 

  12. Clarke DJ . Prader-Willi syndrome and psychoses. Br J Psychiatry 1993; 163: 680–684.

    CAS  PubMed  Google Scholar 

  13. Soni S, Whittington J, Holland AJ, Webb T, Maina EN, Boer H et al. The phenomenology and diagnosis of psychiatric illness in people with Prader-Willi syndrome. Psychol Med 2008; 38: 1505–1514.

    CAS  PubMed  Google Scholar 

  14. Veltman MW, Thompson RJ, Roberts SE, Thomas NS, Whittington J, Bolton PF . Prader-Willi syndrome--a study comparing deletion and uniparental disomy cases with reference to autism spectrum disorders. Eur Child Adolesc Psychiatry 2004; 13: 42–50.

    PubMed  Google Scholar 

  15. Dimitropoulos A, Schultz RT . Autistic-like symptomatology in Prader-Willi syndrome: a review of recent findings. Curr Psychiatry Rep 2007; 9: 159–164.

    PubMed  Google Scholar 

  16. Cassidy SB . Prader-Willi syndrome. J Med Genet 1997; 34: 917–923.

    CAS  PubMed  PubMed Central  Google Scholar 

  17. Whittington JE, Butler JV, Holland AJ . Changing rates of genetic subtypes of Prader-Willi syndrome in the UK. Eur J Hum Genet 2007; 15: 127–130.

    CAS  PubMed  Google Scholar 

  18. Clayton-Smith J, Laan L . Angelman syndrome: a review of the clinical and genetic aspects. J Med Genet 2003; 40: 87–95.

    CAS  PubMed  PubMed Central  Google Scholar 

  19. Sahoo T, del Gaudio D, German JR, Shinawi M, Peters SU, Person RE et al. Prader-Willi phenotype caused by paternal deficiency for the HBII-85 C/D box small nucleolar RNA cluster. Nat Genet 2008; 40: 719–721.

    CAS  PubMed  PubMed Central  Google Scholar 

  20. Wilkins JF, Haig D . What good is genomic imprinting: the function of parent-specific gene expression. Nat Rev Genet 2003; 4: 359–368.

    CAS  PubMed  Google Scholar 

  21. Haig D, Wharton R . Prader-Willi syndrome and the evolution of human childhood. Am J Hum Biol 2003; 15: 320–329.

    PubMed  Google Scholar 

  22. Bressan FF, De Bem TH, Perecin F, Lopes FL, Ambrosio CE, Meirelles FV et al. Unearthing the roles of imprinted genes in the placenta. Placenta 2009; 30: 823–834.

    CAS  PubMed  Google Scholar 

  23. Keverne EB . Genomic imprinting and the maternal brain. Prog Brain Res 2001; 133: 279–285.

    CAS  PubMed  Google Scholar 

  24. Davies CJ . Why is the fetal allograft not rejected? J Anim Sci 2007; 85: E32–E35.

    CAS  PubMed  Google Scholar 

  25. Holland AJ, Treasure J, Coskeran P, Dallow J, Milton N, Hillhouse E . Measurement of excessive appetite and metabolic changes in Prader-Willi syndrome. Int J Obes Relat Metab Disord 1993; 17: 527–532.

    CAS  PubMed  Google Scholar 

  26. Fieldstone A, Zipf WB, Sarter MF, Berntson GG . Food intake in Prader-Willi syndrome and controls with obesity after administration of a benzodiazepine receptor agonist. Obes Res 1998; 6: 29–33.

    CAS  PubMed  Google Scholar 

  27. Wharton RH, Wang T, Graeme-Cook F, Briggs S, Cole RE . Acute idiopathic gastric dilation with gastric necrosis in individuals with Prader-Willi syndrome. Am J Med Genet 1997; 73: 437–441.

    CAS  PubMed  Google Scholar 

  28. Stevenson DA, Heinemann J, Angulo M, Butler MG, Loker J, Rupe N et al. Gastric rupture and necrosis in Prader-Willi syndrome. J Pediatr Gastroenterol Nutr 2007; 45: 272–274.

    PubMed  PubMed Central  Google Scholar 

  29. Hoffman CJ, Aultman D, Pipes P . A nutrition survey of and recommendations for individuals with Prader-Willi syndrome who live in group homes. J Am Diet Assoc 1992; 92: 823–830, 833.

    CAS  PubMed  Google Scholar 

  30. Duker PC, Nielen M . The use of negative practice for the control of pica behavior. J Behav Ther Exp Psychiatry 1993; 24: 249–253.

    CAS  PubMed  Google Scholar 

  31. Einfeld SL, Kavanagh SJ, Smith A, Evans EJ, Tonge BJ, Taffe J . Mortality in Prader-Willi syndrome. Am J Ment Retard 2006; 111: 193–198.

    PubMed  PubMed Central  Google Scholar 

  32. Schrander-Stumpel CT, Curfs LM, Sastrowijoto P, Cassidy SB, Schrander JJ, Fryns JP . Prader-Willi syndrome: causes of death in an international series of 27 cases. Am J Med Genet A 2004; 124A: 333–338.

    PubMed  Google Scholar 

  33. Tauber M, Diene G, Molinas C, Hebert M . Review of 64 cases of death in children with Prader-Willi syndrome (PWS). Am J Med Genet A 2008; 146: 881–887.

    Google Scholar 

  34. Stevenson DA, Heinemann J, Angulo M, Butler MG, Loker J, Rupe N et al. Deaths due to choking in Prader-Willi syndrome. Am J Med Genet A 2007; 143: 484–487.

    PubMed Central  Google Scholar 

  35. Dudley O, Muscatelli F . Clinical evidence of intrauterine disturbance in Prader-Willi syndrome, a genetically imprinted neurodevelopmental disorder. Early Hum Dev 2007; 83: 471–478.

    PubMed  Google Scholar 

  36. Whittington JE, Butler JV, Holland AJ . Pre-, peri- and postnatal complications in Prader-Willi syndrome in a UK sample. Early Hum Dev 2008; 84: 331–336.

    CAS  PubMed  Google Scholar 

  37. Butler JV, Whittington JE, Holland AJ, McAllister CJ, Goldstone AP . The transition between the phenotypes of Prader-Willi syndrome during infancy and early childhood. Dev Med Child Neuro l 2010; 52: e88–e93.

    Google Scholar 

  38. McCune H . Driscoll DJ Prader-Willi Syndrome. In: Efvall SW, Efvall VK (eds). Paediatric nutrition in chronic diseases and developmental disorders, 2nd edn. Oxford University Press: New York, NY, USA, 2005. pp 128–132.

    Google Scholar 

  39. Goldstone AP, Holland AJ, Hauffa BP, Hokken-Koelega AC, Tauber M . Recommendations for the diagnosis and management of Prader-Willi syndrome. J Clin Endocrinol Metab 2008; 93: 4183–4197.

    CAS  PubMed  Google Scholar 

  40. Hinton EC, Holland AJ, Gellatly MS, Soni S, Owen AM . An investigation into food preferences and the neural basis of food-related incentive motivation in Prader-Willi syndrome. J Intellect Disabil Res 2006; 50: 633–642.

    CAS  PubMed  Google Scholar 

  41. Goodman WK, Price LH, Rasmussen SA, Mazure C, Fleischmann RL, Hill CL et al. The Yale-Brown Obsessive Compulsive Scale. I. Development, use, and reliability. Arch Gen Psychiatry 1989; 46: 1006–1011.

    CAS  PubMed  Google Scholar 

  42. Dimitropoulos A, Feurer ID, Roof E, Stone W, Butler MG, Sutcliffe J et al. Appetitive behavior, compulsivity, and neurochemistry in Prader-Willi syndrome. Ment Retard Dev Disabil Res Rev 2000; 6: 125–130.

    CAS  PubMed  PubMed Central  Google Scholar 

  43. Goldstone AP . The hypothalamus, hormones, and hunger: alterations in human obesity and illness. Prog Brain Res 2006; 153: 57–73.

    CAS  PubMed  Google Scholar 

  44. Eiholzer U, Stutz K, Weinmann C, Torresani T, Molinari L, Prader A . Low insulin, IGF-I and IGFBP-3 levels in children with Prader-Labhart-Willi syndrome. Eur J Pediatr 1998; 157: 890–893.

    CAS  PubMed  Google Scholar 

  45. Schuster DP, Osei K, Zipf WB . Characterization of alterations in glucose and insulin metabolism in Prader-Willi subjects. Metabolism 1996; 45: 1514–1520.

    CAS  PubMed  Google Scholar 

  46. Goldstone AP, Patterson M, Kalingag N, Ghatei MA, Brynes AE, Bloom SR et al. Fasting and postprandial hyperghrelinemia in Prader-Willi syndrome is partially explained by hypoinsulinemia, and is not due to peptide YY3-36 deficiency or seen in hypothalamic obesity due to craniopharyngioma. J Clin Endocrinol Metab 2005; 90: 2681–2690.

    CAS  PubMed  Google Scholar 

  47. Bizzarri C, Rigamonti AE, Giannone G, Berardinelli R, Cella SG, Cappa M et al. Maintenance of a normal meal-induced decrease in plasma ghrelin levels in children with Prader-Willi syndrome. Horm Metab Res 2004; 36: 164–169.

    CAS  PubMed  Google Scholar 

  48. DelParigi A, Tschop M, Heiman ML, Salbe AD, Vozarova B, Sell SM et al. High circulating ghrelin: a potential cause for hyperphagia and obesity in prader-willi syndrome. J Clin Endocrinol Metab 2002; 87: 5461–5464.

    CAS  PubMed  Google Scholar 

  49. Haqq AM, Farooqi IS, O’Rahilly S, Stadler DD, Rosenfeld RG, Pratt KL et al. Serum ghrelin levels are inversely correlated with body mass index, age, and insulin concentrations in normal children and are markedly increased in Prader-Willi syndrome. J Clin Endocrinol Metab 2003; 88: 174–178.

    CAS  PubMed  Google Scholar 

  50. Feigerlova E, Diene G, Conte-Auriol F, Molinas C, Gennero I, Salles JP et al. Hyperghrelinemia precedes obesity in Prader-Willi syndrome. J Clin Endocrinol Metab 2008; 93: 2800–2805.

    CAS  PubMed  Google Scholar 

  51. Erdie-Lalena CR, Holm VA, Kelly PC, Frayo RS, Cummings DE . Ghrelin levels in young children with Prader-Willi syndrome. J Pediatr 2006; 149: 199–204.

    CAS  PubMed  Google Scholar 

  52. Haqq AM, Grambow SC, Muehlbauer M, Newgard CB, Svetkey LP, Carrel AL et al. Ghrelin concentrations in Prader-Willi syndrome (PWS) infants and children: changes during development. Clin Endocrinol (Oxf) 2008; 69: 911–920.

    CAS  Google Scholar 

  53. Tan TM, Vanderpump M, Khoo B, Patterson M, Ghatei MA, Goldstone AP . Somatostatin infusion lowers plasma ghrelin without reducing appetite in adults with Prader-Willi syndrome. J Clin Endocrinol Metab 2004; 89: 4162–4165.

    CAS  PubMed  Google Scholar 

  54. Batterham RL, Cowley MA, Small CJ, Herzog H, Cohen MA, Dakin CL et al. Gut hormone PYY(3-36) physiologically inhibits food intake. Nature 2002; 418: 650–654.

    CAS  PubMed  Google Scholar 

  55. Bizzarri C, Rigamonti AE, Luce A, Cappa M, Cella SG, Berini J et al. Children with Prader-Willi syndrome exhibit more evident meal-induced responses in plasma ghrelin and peptide YY levels than obese and lean children. Eur J Endocrinol 2010; 162: 499–505.

    CAS  PubMed  Google Scholar 

  56. Gimenez-Palop O, Gimenez-Perez G, Mauricio D, Gonzalez-Clemente JM, Potau N, Berlanga E et al. A lesser postprandial suppression of plasma ghrelin in Prader-Willi syndrome is associated with low fasting and a blunted postprandial PYY response. Clin Endocrinol (Oxf) 2007; 66: 198–204.

    CAS  Google Scholar 

  57. Zipf WB, Berntson GG . Characteristics of abnormal food-intake patterns in children with Prader-Willi syndrome and study of effects of naloxone. Am J Clin Nutr 1987; 46: 277–281.

    CAS  PubMed  Google Scholar 

  58. Holland AJ, Treasure J, Coskeran P, Dallow J . Characteristics of the eating disorder in Prader-Willi syndrome: implications for treatment. J Intellect Disabil Res 1995; 39 (Part 5): 373–381.

    PubMed  Google Scholar 

  59. Lindgren AC, Barkeling B, Hagg A, Ritzen EM, Marcus C, Rossner S . Eating behavior in Prader-Willi syndrome, normal weight, and obese control groups. J Pediatr 2000; 137: 50–55.

    CAS  PubMed  Google Scholar 

  60. Hoybye C, Barkeling B, Naslund E, Thoren M, Hellstrom PM . Eating behavior and gastric emptying in adults with Prader-Willi syndrome. Ann Nutr Metab 2007; 51: 264–269.

    CAS  PubMed  Google Scholar 

  61. Holsen LM, Zarcone JR, Brooks WM, Butler MG, Thompson TI, Ahluwalia JS et al. Neural mechanisms underlying hyperphagia in Prader-Willi syndrome. Obesity (Silver Spring) 2006; 14: 1028–1037.

    Google Scholar 

  62. Shapira NA, Lessig MC, He AG, James GA, Driscoll DJ, Liu Y . Satiety dysfunction in Prader-Willi syndrome demonstrated by fMRI. J Neurol Neurosurg Psychiatry 2005; 76: 260–262.

    CAS  PubMed  PubMed Central  Google Scholar 

  63. Hinton EC, Holland AJ, Gellatly MS, Soni S, Patterson M, Ghatei MA et al. Neural representations of hunger and satiety in Prader-Willi syndrome. Int J Obes (Lond) 2006; 30: 313–321.

    CAS  Google Scholar 

  64. Craig AD . How do you feel? Interoception: the sense of the physiological condition of the body. Nat Rev Neurosci 2002; 3: 655–666.

    CAS  PubMed  Google Scholar 

  65. Garner DM, Garfinkel PE, Moldofsky H . Perceptual experiences in anorexia nervosa and obesity. Can Psychiatr Assoc J 1978; 23: 249–263.

    CAS  PubMed  Google Scholar 

  66. Pollatos O, Kurz AL, Albrecht J, Schreder T, Kleemann AM, Schopf V et al. Reduced perception of bodily signals in anorexia nervosa. Eat Behav 2008; 9: 381–388.

    PubMed  Google Scholar 

  67. Dykens EM, Maxwell MA, Pantino E, Kossler R, Roof E . Assessment of hyperphagia in Prader-Willi syndrome. Obesity (Silver Spring) 2007; 15: 1816–1826.

    Google Scholar 

  68. Priano L, Miscio G, Grugni G, Milano E, Baudo S, Sellitti L et al. On the origin of sensory impairment and altered pain perception in Prader-Willi syndrome: a neurophysiological study. Eur J Pain 2009; 13: 829–835.

    PubMed  Google Scholar 

  69. Critchley HD, Wiens S, Rotshtein P, Ohman A, Dolan RJ . Neural systems supporting interoceptive awareness. Nat Neurosci 2004; 7: 189–195.

    CAS  PubMed  Google Scholar 

  70. Lucignani G, Panzacchi A, Bosio L, Moresco RM, Ravasi L, Coppa I et al. GABA A receptor abnormalities in Prader-Willi syndrome assessed with positron emission tomography and [11C]flumazenil. Neuroimage 2004; 22: 22–28.

    PubMed  Google Scholar 

  71. Dagher A . The neurobiology of appetite: hunger as addiction. Int J Obes (Lond) 2009; 33 (Suppl 2): S30–S33.

    Google Scholar 

  72. von Deneen KM, Gold MS, Liu Y . Food Addiction and Cues in Prader Willi Syndrome. J Addict Med 2009; 3: 1–7.

    Google Scholar 

  73. James GA, Gold MS, Liu Y . Interaction of satiety and reward response to food stimulation. J Addict Dis 2004; 23: 23–37.

    PubMed  Google Scholar 

  74. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, 4th edn, Text Revision, Washington, DC, American Psychiatric Association, 2000.

  75. Davison GC, Neale JM, Kring AM (eds). Abnormal psychology 9th edn. Wiley: USA, 2004.

    Google Scholar 

  76. Miller JL, James GA, Goldstone AP, Couch JA, He G, Driscoll DJ et al. Enhanced activation of reward mediating prefrontal regions in response to food stimuli in Prader-Willi syndrome. J Neurol Neurosurg Psychiatry 2007; 78: 615–619.

    PubMed  Google Scholar 

  77. Berthoud HR . Neural control of appetite: cross-talk between homeostatic and non-homeostatic systems. Appetite 2004; 43: 315–317.

    PubMed  Google Scholar 

  78. Dimitropoulos A, Schultz RT . Food-related neural circuitry in Prader-Willi syndrome: response to high- versus low-calorie foods. J Autism Dev Disord 2008; 38: 1642–1653.

    PubMed  Google Scholar 

  79. Holsen LM, Zarcone JR, Chambers R, Butler MG, Bittel DC, Brooks WM et al. Genetic subtype differences in neural circuitry of food motivation in Prader-Willi syndrome. Int J Obes (Lond) 2009; 33: 273–283.

    CAS  Google Scholar 

  80. Lutter M, Nestler EJ . Homeostatic and hedonic signals interact in the regulation of food intake. J Nutr 2009; 139: 629–632.

    CAS  PubMed  PubMed Central  Google Scholar 

  81. Hinton EC, Isles AR, Williams NM, Parkinson JA . Excessive appetitive arousal in Prader-Willi syndrome. Appetite 2010; 54: 225–228.

    CAS  PubMed  Google Scholar 

  82. O’Rahilly S, Farooqi IS . Human obesity as a heritable disorder of the central control of energy balance. Int J Obes (Lond) 2008; 32 (Suppl 7): S55–S61.

    Google Scholar 

  83. Farooqi IS, Yeo GS, Keogh JM, Aminian S, Jebb SA, Butler G et al. Dominant and recessive inheritance of morbid obesity associated with melanocortin 4 receptor deficiency. J Clin Invest 2000; 106: 271–279.

    CAS  PubMed  PubMed Central  Google Scholar 

  84. Walters RG, Jacquemont S, Valsesia A, de Smith AJ, Martinet D, Andersson J et al. A new highly penetrant form of obesity due to deletions on chromosome 16p11.2. Nature 2010; 463: 671–675.

    CAS  PubMed  PubMed Central  Google Scholar 

  85. Swaab DF, Purba JS, Hofman MA . Alterations in the hypothalamic paraventricular nucleus and its oxytocin neurons (putative satiety cells) in Prader-Willi syndrome: a study of five cases. J Clin Endocrinol Metab 1995; 80: 573–579.

    CAS  PubMed  Google Scholar 

  86. Heinrichs M, Domes G . Neuropeptides and social behaviour: effects of oxytocin and vasopressin in humans. Prog Brain Res 2008; 170: 337–350.

    CAS  PubMed  Google Scholar 

  87. Holland A, Whittington J, Hinton E . The paradox of Prader-Willi syndrome: a genetic model of starvation. Lancet 2003; 362: 989–991.

    CAS  PubMed  Google Scholar 

  88. Hales CN, Barker DJ . Type 2 (non-insulin-dependent) diabetes mellitus: the thrifty phenotype hypothesis. Diabetologia 1992; 35: 595–601.

    CAS  PubMed  Google Scholar 

  89. Hales CN, Barker DJ, Clark PM, Cox LJ, Fall C, Osmond C et al. Fetal and infant growth and impaired glucose tolerance at age 64. BMJ 1991; 303: 1019–1022.

    CAS  PubMed  PubMed Central  Google Scholar 

  90. Barker DJ, Hales CN, Fall CH, Osmond C, Phipps K, Clark PM . Type 2 (non-insulin-dependent) diabetes mellitus, hypertension and hyperlipidaemia (syndrome X): relation to reduced fetal growth. Diabetologia 1993; 36: 62–67.

    CAS  PubMed  Google Scholar 

  91. Barker DJ . Obesity and early life. Obes Rev 2007; 8: (Suppl 1): 45-49.

    Google Scholar 

  92. Tanaka M, Naruo T, Yasuhara D, Tatebe Y, Nagai N, Shiiya T et al. Fasting plasma ghrelin levels in subtypes of anorexia nervosa. Psychoneuroendocrinology 2003; 28: 829–835.

    CAS  PubMed  Google Scholar 

  93. Whittington JE, Holland AJ (eds). Prader-Willi Syndrome: Development and Manifestations. Cambridge University Press: Cambridge, UK, 2004.

    Google Scholar 

  94. Skryabin BV, Gubar LV, Seeger B, Pfeiffer J, Handel S, Robeck T et al. Deletion of the MBII-85 snoRNA gene cluster in mice results in postnatal growth retardation. PLoS Genet 2007; 3: e235.

    PubMed  PubMed Central  Google Scholar 

  95. Stefan M, Claiborn KC, Stasiek E, Chai JH, Ohta T, Longnecker R et al. Genetic mapping of putative Chrna7 and Luzp2 neuronal transcriptional enhancers due to impact of a transgene-insertion and 6.8 Mb deletion in a mouse model of Prader-Willi and Angelman syndromes. BMC Genomics 2005; 6: 157.

    PubMed  PubMed Central  Google Scholar 

  96. Forhead AJ, Fowden AL . The hungry fetus? Role of leptin as a nutritional signal before birth. J Physiol 2009; 587: 1145–1152.

    CAS  PubMed  PubMed Central  Google Scholar 

  97. Farooqi IS, O’Rahilly S . Leptin: a pivotal regulator of human energy homeostasis. Am J Clin Nutr 2009; 89: 980S–984S.

    CAS  PubMed  Google Scholar 

  98. Berthoud HR . Vagal and hormonal gut-brain communication: from satiation to satisfaction. Neurogastroenterol Motil 2008; 20 (Suppl 1): 64–72.

    CAS  PubMed  PubMed Central  Google Scholar 

  99. Farooqi IS, Jebb SA, Langmack G, Lawrence E, Cheetham CH, Prentice AM et al. Effects of recombinant leptin therapy in a child with congenital leptin deficiency. N Engl J Med 1999; 341: 879–884.

    CAS  PubMed  Google Scholar 

  100. Farooqi IS, Matarese G, Lord GM, Keogh JM, Lawrence E, Agwu C et al. Beneficial effects of leptin on obesity, T cell hyporesponsiveness, and neuroendocrine/metabolic dysfunction of human congenital leptin deficiency. J Clin Invest 2002; 110: 1093–1103.

    CAS  PubMed  PubMed Central  Google Scholar 

  101. Farooqi IS, Bullmore E, Keogh J, Gillard J, O'Rahilly S, Fletcher PC . Leptin regulates striatal regions and human eating behavior. Science 2007; 317: 1355.

    CAS  PubMed  Google Scholar 

  102. Shapira NA, Lessig MC, Lewis MH, Goodman WK, Driscoll DJ . Effects of topiramate in adults with Prader-Willi syndrome. Am J Ment Retard 2004; 109: 301–309.

    PubMed  Google Scholar 

  103. Papavramidis ST, Kotidis EV, Gamvros O . Prader-Willi syndrome-associated obesity treated by biliopancreatic diversion with duodenal switch. Case report and literature review. J Pediatr Surg 2006; 41: 1153–1158.

    PubMed  Google Scholar 

  104. Scheimann AO, Butler MG, Gourash L, Cuffari C, Klish W . Critical analysis of bariatric procedures in Prader-Willi syndrome. J Pediatr Gastroenterol Nutr 2008; 46: 80–83.

    CAS  PubMed  PubMed Central  Google Scholar 

  105. Eiholzer U, L’Allemand D, Schlumpf M, Rousson V, Gasser T, Fusch C . Growth hormone and body composition in children younger than 2 years with Prader-Willi syndrome. J Pediatr 2004; 144: 753–758.

    CAS  PubMed  Google Scholar 

  106. Soni S, Whittington J, Holland AJ, Webb T, Maina E, Boer H et al. The course and outcome of psychiatric illness in people with Prader-Willi syndrome: implications for management and treatment. J Intellect Disabil Res 2007; 51: 32–42.

    CAS  PubMed  Google Scholar 

  107. Woodcock KA, Oliver C, Humphreys GW . A specific pathway can be identified between genetic characteristics and behaviour profiles in Prader-Willi syndrome via cognitive, environmental and physiological mechanisms. J Intellect Disabil Res 2009; 53: 493–500.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to C J McAllister.

Ethics declarations

Competing interests

The authors declare no conflict of interest.

Rights and permissions

Reprints and permissions

About this article

Cite this article

McAllister, C., Whittington, J. & Holland, A. Development of the eating behaviour in Prader–Willi Syndrome: advances in our understanding. Int J Obes 35, 188–197 (2011). https://doi.org/10.1038/ijo.2010.139

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/ijo.2010.139

Keywords

This article is cited by

Search

Quick links