Sylvian fissure morphology in Prader-Willi syndrome and early-onset morbid obesity

التفاصيل البيبلوغرافية
العنوان: Sylvian fissure morphology in Prader-Willi syndrome and early-onset morbid obesity
المؤلفون: Miller, Jennifer L, Couch, Jessica A, Leonard, Christiana M, Schwenk, Krista, Towler, Stephen D, Shuster, Jonathan, Goldstone, Anthony P, He, Guojun, Driscoll, Daniel J, Liu, Yijun
المصدر: Genetics in Medicine; August 2007, Vol. 9 Issue: 8 p536-543, 8p
مستخلص: 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.
قاعدة البيانات: Supplemental Index
الوصف
تدمد:10983600
15300366
DOI:10.1097/GIM.0b013e31812f720d