Journal article
Abnormal brain structure in adults with Van der Woude syndrome
Clinical genetics, Vol.71(6), pp.511-517
06/2007
DOI: 10.1111/j.1399-0004.2007.00799.x
PMID: 17539900
Abstract
Van der Woude syndrome (VWS) is an autosomal dominant disorder manifested in cleft lip and/or palate and lip pits. Isolated clefts of the lip and/or palate (ICLP) have both genotype and phenotype overlap with VWS. Subjects with ICLP have abnormalities in brain structure and function. Given the similarities between VWS and ICLP, the current study was designed to evaluate the pattern of brain structure of adults with VWS. Fourteen adults with VWS were compared to age- and gender-matched healthy controls. Brain structure was evaluated using magnetic resonance imaging. All subjects with VWS had enlarged volumes of the anterior regions of the cerebrum. Men with VWS had reduced volumes of the posterior cerebrum. Anterior cerebrum volume was negatively correlated with intelligent quotient in the subjects with VWS indicating that the enlargement of this brain region was 'pathologic.' The pattern of brain structure in VWS is nearly identical to those seen in ICLP. In addition, men are affected more severely. Pathologic enlargement of the tissue and a gender effect with men affected more severely are common features of neurodevelopmental disorders supporting the notion that the brain structure of VWS and ICLP may be because of abnormal brain development.
Details
- Title: Subtitle
- Abnormal brain structure in adults with Van der Woude syndrome
- Creators
- P Nopoulos - Department of Psychiatry, University of Iowa College of Medicine, IA 52242, USA. peggy-nopoulos@uiowa.eduL RichmanN C AndreasenJ C MurrayB Schutte
- Resource Type
- Journal article
- Publication Details
- Clinical genetics, Vol.71(6), pp.511-517
- DOI
- 10.1111/j.1399-0004.2007.00799.x
- PMID
- 17539900
- NLM abbreviation
- Clin Genet
- ISSN
- 0009-9163
- eISSN
- 1399-0004
- Publisher
- Denmark
- Grant note
- 5 K23 DE000417-05 / NIDCR NIH HHS MH40856 / NIMH NIH HHS MH60990 / NIMH NIH HHS MH031593 / NIMH NIH HHS
- Language
- English
- Date published
- 06/2007
- Academic Unit
- Neurology; Psychiatry; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Epidemiology; Iowa Neuroscience Institute; Pediatric Dentistry; Craniofacial Anomalies Research Center; Dental Research
- Record Identifier
- 9984003998702771
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