Sylvian fissure morphology in Prader-Willi syndrome and early-onset morbid obesity.
OBJECTIVE
Prader-Willi syndrome is a well-defined genetic cause of childhood-onset obesity that can serve as a model for investigating early-onset childhood obesity. Individuals with Prader-Willi syndrome have speech and language impairments, suggesting possible involvement of the perisylvian region of the brain. Clinical observations suggest that many individuals with early-onset morbid obesity have similar speech/language deficits, indicating possible perisylvian involvement in these children as well. We hypothesized that similar perisylvian abnormalities may exist in both disorders.


METHODS
Participants included individuals with Prader-Willi syndrome (n = 27), their siblings (n = 16), individuals with early-onset morbid obesity (n = 13), and their siblings (n = 10). Quantitative and qualitative assessments of sylvian fissure conformation, insula closure, and planum temporale length were performed blind to hemisphere and diagnosis.


RESULTS
Quantitative measurements verified incomplete closure of the insula in individuals with Prader-Willi syndrome. Planar asymmetry showed its normal bias toward leftward asymmetry in all groups except those with Prader-Willi syndrome maternal uniparental disomy. Individuals with Prader-Willi syndrome and siblings had a normal distribution of sylvian fissure types in both hemispheres, while individuals with early-onset morbid obesity and their siblings had a high proportion of rare sylvian fissures in the right hemisphere.


CONCLUSIONS
The contrast between the anatomic findings in individuals with Prader-Willi syndrome and early-onset morbid obesity suggests that the language problems displayed by children with these two conditions may be associated with different neurodevelopmental processes.
Miller
Jennifer L
JL
Departments of Pediatrics, University of Florida, College of Medicine, Gainesville, Florida 32610-0296, USA. millejl@peds.ufl.edu
Couch
Jessica A
JA
Leonard
Christiana M
CM
Schwenk
Krista
K
Towler
Stephen D
SD
Shuster
Jonathan
J
Goldstone
Anthony P
AP
He
Guojun
G
Driscoll
Daniel J
DJ
Liu
Yijun
Y
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001921:Brain / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009767:Obesity, Morbid / Q000209:etiology / Q000235:genetics / Q000473:pathology*; D011218:Prader-Willi Syndrome / Q000235:genetics / Q000473:pathology*