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Prader-Willi Syndrome and Eating and Weight Disorders

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Hidden and Lesser-known Disordered Eating Behaviors in Medical and Psychiatric Conditions

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Abstract

Prader-Willi syndrome (PWS) is the most common form of compulsive hyperphagia and obesity on a genetic basis. Together with Angelman syndrome, PWS was the first genomic imprinting disorder described in humans.

Starting from John Langdon Down’s observations around the E.C. case, some historical notes introduce the chapter that addresses the following topics:

  • Epidemiological and genetic data dwelling on the concept of imprinted genes

  • Somatic characters and medical comorbidities

  • Fertility and mortality

  • Neurocognitive development and psychosocial adaptation

  • Evolutions in eating behavior and body weight in the natural history of the syndrome

  • Psychiatric symptoms that most often appear in individuals with PWS

  • Does it make sense to talk about psychiatric comorbidity in PWS? Research Domain Criteria are an attempt to integrate and overcome categorical classifications of mental disorders

  • Brief mention of Angelman syndrome and the PWS phenotype of fragile X syndrome

  • The therapy is still insufficient in results. Recent guidelines for clinical practice recommend timely diagnosis, constant family support, and multidisciplinary treatment. Several dietary strategies have been attempted to combat obesity. Bariatric surgery is not infrequently used, even in adolescents, but there is still a lack of convincing data. Deep brain stimulation has been tested experimentally, with damage and without benefits.

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  1. University of Roma Sapienza, Roma, Italy

    Massimo Cuzzolaro

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  1. Private Hospital "Salus" of Ferrara, University of Ferrara, Ferrara, Italy

    Emilia Manzato

  2. Formerly Sapienza University of Rome, Founding Editor of Eating and Weight Disorders, Campiglia Marittima, Livorno, Italy

    Massimo Cuzzolaro

  3. Experimental Medicine Department, Sapienza University of Rome, Editor-in-Chief of Eating and Weight Disorders, Roma, Roma, Italy

    Lorenzo Maria Donini

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Cuzzolaro, M. (2022). Prader-Willi Syndrome and Eating and Weight Disorders. In: Manzato, E., Cuzzolaro, M., Donini, L.M. (eds) Hidden and Lesser-known Disordered Eating Behaviors in Medical and Psychiatric Conditions . Springer, Cham. https://doi.org/10.1007/978-3-030-81174-7_33

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