Regional cerebral glucose metabolic abnormality in Prader-Willi syndrome: A 18F-FDG PET study under sedation.
Prader-Willi syndrome (PWS) is a genetic disorder caused by the nonexpression of paternal genes in the PWS region of chromosome 15q11-13 and is the most common cause of human syndromic obesity.


METHODS
We investigated regional brain metabolic impairment in children with PWS by 18F-FDG PET. Sixteen children with PWS (9 males, 7 females; mean age +/- SD, 4.2 +/- 1.1 y) and 7 healthy children (4 males, 3 females; mean age +/- SD, 4.0 +/- 1.7 y) underwent brain 18F-FDG PET in the resting state. The images of PWS children were compared using statistical parametric mapping analysis with those of healthy children in a voxelwise manner.


RESULTS
Group comparison showed that children with PWS had decreased glucose metabolism in the right superior temporal gyrus and left cerebellar vermis, regions that are associated with taste perception/food reward and cognitive and emotional function, respectively. Metabolism was increased in the right orbitofrontal, bilateral middle frontal, right inferior frontal, left superior frontal, and bilateral anterior cingulate gyri, right temporal pole, and left uncus, regions that are involved in cognitive functions related to eating or obsessive-compulsive behavior. Interestingly, no significant metabolic abnormality was found in the hypothalamus, the brain region believed to be most involved in energy intake and expenditure.


CONCLUSIONS
This study describes the neural substrate underlying the abnormal eating behavior and psychobehavioral problems of PWS.
Kim
Sang Eun
SE
Department of Nuclear Medicine, Seoul National University College of Medicine, Seoul, Korea.
Jin
Dong-Kyu
DK
Cho
Sang Soo
SS
Kim
Ji-Hae
JH
Hong
Sungdo David
SD
Paik
Kyung Hoon
KH
Oh
Yoo Joung
YJ
Kim
An Hee
AH
Kwon
Eun Kyung
EK
Choe
Yon Ho
YH
D000758:Anesthesia; D001921:Brain / Q000378:metabolism* / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D003952:Diagnostic Imaging / Q000379:methods; D005260:Female; D019788:Fluorodeoxyglucose F18 / Q000494:pharmacology*; D005947:Glucose / Q000378:metabolism*; D006801:Humans; D008297:Male; D009683:Nuclear Medicine / Q000379:methods*; D011218:Prader-Willi Syndrome / Q000378:metabolism / Q000473:pathology*; D019275:Radiopharmaceuticals / Q000494:pharmacology*
D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18; D005947:Glucose