Neurocognitive findings in Prader-Willi syndrome and early-onset morbid obesity.
OBJECTIVE
To examine whether early-onset morbid obesity is associated with cognitive impairment, neuropathologic changes, and behavioral problems.


METHODS
This case-control study compared head MRI scans and cognitive, achievement, and behavioral evaluations of subjects with Prader-Willi syndrome (PWS), early-onset morbid obesity (EMO), and normal-weight sibling control subjects from both groups. Head MRI was done on 17 PWS, 18 EMO, and 21 siblings, and cognitive, achievement, and behavioral evaluations were done on 19 PWS, 17 EMO, and 24 siblings.


RESULTS
The mean General Intellectual Ability score of the EMO group was 77.4 +/- 17.8; PWS, 63.3 +/- 14.2; and control subjects, 106.4 +/- 13.0. Achievement scores for the three groups were EMO, 78.7 +/- 18.8; PWS, 71.2 +/- 17.0; and control subjects, 104.8 +/- 17.0. Significant negative behaviors and poor adaptive skills were found in the EMO group. White matter lesions were noted on brain MRI in 6 subjects with PWS and 5 with EMO. None of the normal-weight control subjects had these findings.


CONCLUSIONS
Individuals with EMO have significantly lower cognitive function and more behavioral problems than control subjects with no history of childhood obesity. Both EMO and PWS subjects have white matter lesions on brain MRI that have not previously been described.
Miller
Jennifer
J
Department of Pediatrics and the Center for Mammalian Genetics, University of Florida, College of Medicine, Gainesville, Florida 32610-0296, USA.
Kranzler
John
J
Liu
Yijun
Y
Schmalfuss
Ilona
I
Theriaque
Douglas W
DW
Shuster
Jonathan J
JJ
Hatfield
Ann
A
Mueller
O Thomas
OT
Goldstone
Anthony P
AP
Sahoo
Trilochan
T
Beaudet
Arthur L
AL
Driscoll
Daniel J
DJ
D000124:Achievement; D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000886:Anthropometry; D001921:Brain / Q000473:pathology / Q000503:physiopathology*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D002872:Chromosome Deletion; D002884:Chromosomes, Human, Pair 15 / Q000235:genetics; D003072:Cognition Disorders / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D009767:Obesity, Morbid / Q000453:epidemiology* / Q000235:genetics; D011218:Prader-Willi Syndrome / Q000453:epidemiology* / Q000235:genetics; D044105:Receptor, Melanocortin, Type 4 / Q000235:genetics; D012720:Severity of Illness Index; D012959:Socioeconomic Factors
C470072:MC4R protein, human; D044105:Receptor, Melanocortin, Type 4