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

Jennifer L Miller1, Jessica A Couch, Christiana M Leonard

  • 1Departments of Pediatrics, University of Florida, College of Medicine, Gainesville, Florida 32610-0296, USA. millejl@peds.ufl.edu

Genetics in Medicine : Official Journal of the American College of Medical Genetics
|August 21, 2007
PubMed

Insights

Individuals with Prader-Willi syndrome (PWS) and early-onset morbid obesity (EOMO) show distinct brain structure differences. These findings suggest different neurodevelopmental origins for speech and language impairments in PWS and EOMO.

Area of Science:

  • Neuroscience
  • Genetics
  • Developmental Pediatrics

Background:

  • Prader-Willi syndrome (PWS) is a genetic disorder causing childhood obesity and speech impairments.
  • Early-onset morbid obesity (EOMO) in children may share speech/language deficits with PWS.
  • The perisylvian region of the brain is implicated in speech and language processing.

Purpose of the Study:

  • To investigate potential similarities in perisylvian brain abnormalities between PWS and EOMO.
  • To compare neurodevelopmental processes underlying speech and language impairments in these conditions.

Main Methods:

  • Quantitative and qualitative assessments of sylvian fissure conformation, insula closure, and planum temporale length.
  • Participants included individuals with PWS (n=27), their siblings (n=16), individuals with EOMO (n=13), and their siblings (n=10).
  • Assessments were performed blind to hemisphere and diagnosis.

Main Results:

  • Incomplete insula closure was verified in individuals with PWS.
  • Leftward asymmetry bias was altered in PWS maternal uniparental disomy.
  • Individuals with EOMO and siblings showed a high proportion of rare sylvian fissures in the right hemisphere, unlike PWS groups.

Conclusions:

  • Anatomic findings differ significantly between PWS and EOMO.
  • Speech and language problems in PWS and EOMO may arise from distinct neurodevelopmental processes.
  • This study highlights unique neuroanatomical profiles associated with these conditions.
Abstract

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