Cognitive dysfunction in adults with Van der Woude syndrome

Peg Nopoulos; Lynn Richman; Nancy Andreasen; Jeffrey C Murray; Brian Schutte

doi:10.1097/GIM.0b013e3180335abd

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Cognitive dysfunction in adults with Van der Woude syndrome

Journal article

Open access

Peer reviewed

Cognitive dysfunction in adults with Van der Woude syndrome

Peg Nopoulos, Lynn Richman, Nancy Andreasen, Jeffrey C Murray and Brian Schutte

Genetics in medicine, Vol.9(4), pp.213-218

04/2007

DOI: 10.1097/GIM.0b013e3180335abd

PMID: 17438385

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https://doi.org/10.1097/GIM.0b013e3180335abdView

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Abstract

Van der Woude syndrome is an autosomal dominant disorder manifested in clefts of the lip and/or palate and lip pits. There is phenotypic and genotypic overlap between Van der Woude syndrome and isolated cleft of the lip and/or palate. Subjects with isolated cleft of the lip and/or palate have been shown to have cognitive dysfunction. Given the similarities between Van der Woude syndrome and isolated cleft of the lip and/or palate, the current study was designed to evaluate the pattern of cognitive function in adults with Van der Woude syndrome. Fourteen adults with Van der Woude syndrome were compared with age- and gender-matched controls. A battery of cognitive tests was administered to determine general IQ as well as more specific cognitive performance measures. All subjects with Van der Woude syndrome showed deficits in performance on an executive function task. In addition, males with Van der Woude syndrome had significantly lower scores on intelligence measures and on a verbal fluency task compared with controls. The pattern of cognitive function in Van der Woude syndrome is very similar to that seen in isolated cleft of the lip and/or palate. In addition, the findings have a significant gender effect in which males are more severely affected than females. This pattern is common to other conditions with a neurodevelopmental etiology, supporting the notion that the cognitive deficits of Van der Woude syndrome and isolated cleft of the lip and/or palate are due to abnormal brain development.

Cleft Palate - diagnosis

Lip - abnormalities

Cleft Lip - diagnosis

Humans

Male

Cleft Palate - genetics

Cognition Disorders - genetics

Brain - abnormalities

Syndrome

Cognition Disorders - diagnosis

Cleft Lip - genetics

Speech Disorders - etiology

Genes, Dominant

Cognition Disorders - etiology

Adult

Female

Details

Title: Subtitle: Cognitive dysfunction in adults with Van der Woude syndrome
Creators: Peg Nopoulos - Department of Psychiatry, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA. peggy-nopoulos@uiowa.edu
Lynn Richman
Nancy Andreasen
Jeffrey C Murray
Brian Schutte
Resource Type: Journal article
Publication Details: Genetics in medicine, Vol.9(4), pp.213-218
DOI: 10.1097/GIM.0b013e3180335abd
PMID: 17438385
NLM abbreviation: Genet Med
ISSN: 1098-3600
eISSN: 1530-0366
Publisher: United States
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: 04/2007
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: 9984003422802771

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