PET scan perfusion imaging in the Prader-Willi syndrome: new insights into the psychiatric and social disturbances

J Cereb Blood Flow Metab. 2011 Jan;31(1):275-82. doi: 10.1038/jcbfm.2010.87. Epub 2010 Jun 30.

Abstract

The Prader-Willi syndrome (PWS), a rare multisystem genetic disease, leads to severe disabilities, such as morbid obesity, endocrine dysfunctions, psychiatric disorders, and social disturbances. We explored the whole brain of patients with PWS to detect abnormalities that might explain the behavioral and social disturbances, as well as the psychiatric disorders of these patients. Nine patients with PWS (six males, three females; mean age 16.4 years) underwent a positron emission tomography (PET) scan with H(2)(15)O as a tracer to measure regional cerebral blood flow (rCBF). The images were compared with those acquired from nine controls (six males, three females; mean age 21.2 years). A morphologic magnetic resonance imaging (MRI) was also performed in PWS patients, and their cognitive and behavioral skills were assessed with Wechsler Intelligence Scale for Children III and the Child Behavior Check List (CBCL). The MRI images showed no evident anatomic abnormalities, whereas PET scans revealed hypoperfused brain regions in PWS patients compared with controls, particularly in the anterior cingulum and superior temporal regions. We observed a significant relationship (P<0.05) between rCBF in the hypoperfused regions and CBCL scores. The functional consequences of these perfusion abnormalities in specific brain regions might explain the behavioral and social problems observed in these individuals.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Cerebrovascular Circulation / physiology
  • Cerebrovascular Disorders / diagnostic imaging
  • Cerebrovascular Disorders / etiology
  • Child
  • Child Behavior Disorders / etiology
  • Child Behavior Disorders / psychology
  • Female
  • Humans
  • Image Processing, Computer-Assisted
  • Intelligence Tests
  • Magnetic Resonance Imaging
  • Male
  • Mental Disorders / diagnostic imaging*
  • Mental Disorders / etiology
  • Mental Disorders / psychology*
  • Neuropsychological Tests
  • Positron-Emission Tomography
  • Prader-Willi Syndrome / diagnostic imaging*
  • Prader-Willi Syndrome / psychology*
  • Psychotropic Drugs / therapeutic use
  • Regression, Psychology
  • Social Behavior Disorders / diagnostic imaging*
  • Social Behavior Disorders / etiology
  • Social Behavior Disorders / psychology*
  • Young Adult

Substances

  • Psychotropic Drugs