title: Weight control and behavior rehabilitation in a patient suffering from Prader Willi syndrome.
abstract: BACKGROUND
  This study reports a case of Prader Willi syndrome (PWS), a genomic imprinting disease related to chromosome regions 15q11.2-q13 15, which includes hypothalamic dysfunction leading to hyperphagia, obesity, shortness, sleep abnormalities. Our case is extremely severe, in comparison to other PWS cases described in literature, due to the association with severe emotional and psychiatric symptoms: oppositional behaviour, rigidity of thought, skin picking and pathological hoarding.
  
  
  METHODS
  We described the case of a Caucasian male patient suffering from PWS, treated in outpatient care by local Mental Health Centre and supported by Social Service, who was admitted to a residential rehabilitative facility. After a 2-year follow-up, the patient showed a global improvement in symptoms and functioning, as registered by the rating scales administered. At the end of observation period, we also reported an important improvement in weight control, reducing the risk of obesity and related diseases, therefore improving the prognosis of life.
  
  
  CONCLUSIONS
  This case highlights the need for long-term, individualized and multi-professional treatment in patients suffering from a complex genetic syndrome with both organic and psychological alterations, for which medical care setting and pharmacological treatments are not sufficient. Clinical observation of this case leads us to compare PWS to drug addiction and indirectly endorse the neurophysiological hypothesis that food and drugs stimulate the same brain circuits in the limbic system.
authors.affiliation: Psychiatrist, SPDC, Department of Mental Health, Az-USL-Modena, Via P. Giardini, 1355 Baggiovara, 41126, Modena, Italy. saradilorenzo1@alice.it.
authors.forename: Rosaria
authors.initials: R
authors.lastname: Di Lorenzo
authors.affiliation: Registered Nurse, School of Nursing, University of Modena and Reggio Emilia, Via del Pozzo, 71, 41124, Modena, Italy.
authors.forename: Sara
authors.initials: S
authors.lastname: Sberveglieri
authors.affiliation: Psychiatrist, Outpatient Care Service, Department of Mental Health, Az-USL-Modena, Via I. Newton, 150, 41121, Modena, Italy.
authors.forename: Donatella
authors.initials: D
authors.lastname: Marrama
authors.affiliation: Resident in Psychiatry, School of Psychiatry, University of Modena and Reggio Emilia, Via del Pozzo, 71, 41124, Modena, Italy.
authors.forename: Giulia
authors.initials: G
authors.lastname: Landi
authors.affiliation: Nurse Researcher, School of Nursing, University of Modena and Reggio Emilia, Via del Pozzo, 71, 41124, Modena, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Ferri
mesh_terms: D001519:Behavior; D015992:Body Mass Index; D001835:Body Weight; D006760:Hospitalization; D006801:Humans; D011218:Prader-Willi Syndrome / Q000523:psychology / Q000534:rehabilitation*; D016896:Treatment Outcome
keywords: Aggressive behaviour; Genomic imprinting; Hyperphagia and obesity; Hypothalamic dysfunction; Long-term individualized rehabilitation