title: PET scan perfusion imaging in the Prader-Willi syndrome: new insights into the psychiatric and social disturbances.
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.
authors.affiliation: INSERM, Imagerie cérébrale et handicaps neurologiques UMR 825, Toulouse, France.
authors.forename: Carine
authors.initials: C
authors.lastname: Mantoulan
authors.forename: Pierre
authors.initials: P
authors.lastname: Payoux
authors.forename: Gwenaëlle
authors.initials: G
authors.lastname: Diene
authors.forename: Mélanie
authors.initials: M
authors.lastname: Glattard
authors.forename: Bernadette
authors.initials: B
authors.lastname: Rogé
authors.forename: Catherine
authors.initials: C
authors.lastname: Molinas
authors.forename: Annick
authors.initials: A
authors.lastname: Sevely
authors.forename: Monica
authors.initials: M
authors.lastname: Zilbovicius
authors.forename: Pierre
authors.initials: P
authors.lastname: Celsis
authors.forename: Maïthé
authors.initials: M
authors.lastname: Tauber
mesh_terms: D000293:Adolescent; D002560:Cerebrovascular Circulation / Q000502:physiology; D002561:Cerebrovascular Disorders / Q000000981:diagnostic imaging / Q000209:etiology; D002648:Child; D002653:Child Behavior Disorders / Q000209:etiology / Q000523:psychology; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007361:Intelligence Tests; D008279:Magnetic Resonance Imaging; D008297:Male; D001523:Mental Disorders / Q000000981:diagnostic imaging* / Q000209:etiology / Q000523:psychology*; D009483:Neuropsychological Tests; D049268:Positron-Emission Tomography; D011218:Prader-Willi Syndrome / Q000000981:diagnostic imaging* / Q000523:psychology*; D011619:Psychotropic Drugs / Q000627:therapeutic use; D012043:Regression, Psychology; D012920:Social Behavior Disorders / Q000000981:diagnostic imaging* / Q000209:etiology / Q000523:psychology*; D055815:Young Adult
chemical_list: D011619:Psychotropic Drugs