Psychopathological disorders in Prader-Willi syndrome

Endocrinol Diabetes Nutr (Engl Ed). 2019 Nov;66(9):579-587. doi: 10.1016/j.endinu.2019.03.004. Epub 2019 Apr 18.
[Article in English, Spanish]

Abstract

Prader-Willi syndrome is a genetic disorder caused by chromosomal changes in segment 15q11-q13 including cognitive, mental, and behavioral symptoms, as well as a specific physical phenotype. Both the most common psychopathological changes (intellectual disability, obsessions, impulsivity, autism spectrum disorders, self-injuries) and the main psychiatric comorbidities (affective disorders, psychosis, obsessive-compulsive disorder, autism spectrum disorder) are characterized by a great heterogeneity, which warrants the need for better identification of their frequency and clinical signs. In addition to its effects on body compositionand hypotony, growth hormone has been shown to be useful for regulating patient behavior, and psychoactive drugs are also an option. Other alternatives have shown promising results in experimental trials. Adequate understanding of the psychopathology associated to Prader-Willi syndrome would allow for improving clinical approach, symptom identification, detection of comorbidities, and administration of more effective treatments, leading to better clinical outcomes.

Keywords: Comorbidity; Comorbilidad; Prader-Willi syndrome; Psicopatología; Psychopathology; Síndrome de Prader-Willi.

Publication types

  • Review

MeSH terms

  • Antipsychotic Agents / therapeutic use
  • Attention Deficit Disorder with Hyperactivity / psychology
  • Autism Spectrum Disorder / psychology
  • Child
  • Cognition Disorders / psychology
  • Comorbidity
  • Emotional Regulation
  • Food Addiction / drug therapy
  • Food Addiction / psychology
  • Humans
  • Intellectual Disability / psychology
  • Mood Disorders / drug therapy
  • Mood Disorders / psychology
  • Obsessive-Compulsive Disorder / drug therapy
  • Obsessive-Compulsive Disorder / psychology
  • Phenotype
  • Prader-Willi Syndrome / drug therapy
  • Prader-Willi Syndrome / genetics
  • Prader-Willi Syndrome / psychology*
  • Self-Injurious Behavior / drug therapy
  • Self-Injurious Behavior / psychology
  • Sleep Wake Disorders / psychology

Substances

  • Antipsychotic Agents