Journal
GENETICS IN MEDICINE
Volume 9, Issue 8, Pages 536-543Publisher
NATURE PUBLISHING GROUP
DOI: 10.1097/GIM.0b013e31812f720d
Keywords
Prader-Willi syndrome; early-onset obesity; sylvian fissure; speech; language
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Funding
- Medical Research Council [MC_U120061305] Funding Source: Medline
- NCRR NIH HHS [MO1-RR00082, U54 RR019478] Funding Source: Medline
- NICHD NIH HHS [K24 HD01361, 1F32HD49261-01] Funding Source: Medline
- Medical Research Council [MC_U120061305] Funding Source: researchfish
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Purpose: 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.
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